whatisthefirstenzymeandstepinethanol

metabolism?secondstep?whatisacofactorin
bothofthesesteps?whatvitamindoesthiscome
from?

alcoholdehyrogenase(alcoholto
acetaldehyde);acetaldehydedehydrogenase
(acetaldehydetoacetate);NAD+;B3(niacin)

whichistheratelimitingenzymeinethanolmetabolism?whereis
thisenzymelocated?whereistheotherenzymelocated?whatis
thelimitingreagent?

whatkindofkineticsdoesalcoholdehydrogenase
workunder?whataretheimplicationsofthis?

alcoholdehydrogenase;
cytosol;mitochondria;NAD+

zeroorder;increasedproductionofNADHno
mattertheamountofendproductsproduced

whatdrugblocksalcoholdehydrogenase?
whatisitusedfor?

fomepizole;antidoteformethanolorethylene
glycolpoisoning

whatdrugblocksacetaldehydedehyrogenase?
whatareitssideeffects?

disulfiram(antabuse);acetaldehydeaccumulate
contributingtohangover

whatarethethreeresults
thatcomefromincreased
NADHinalcohol
metabolism?

pyruvatetolactate(resultsinlacticacidosisanddecreasedpyruvate
foroxaloacetateforgluconeogenesis),increasedproductionof
glycerol3phosphatefromDHAPforTGsynthesis;andincreased
betahydroxybutyrateketoacidproductionfromincreasedacetate

whatcontributestotheacidosisinalcoholism?

whatthreethingscontribute
tofattychangeintheliverin
alcoholism?

whatcauses
hypoglycemiain
alcoholism?

whatcausesgoutin
alcoholism?

lacticacidosisandketoacidosis

increasedG3PforTGsynthesis,increasedacetylCoA(Fattyacid
synthesisforTGsynthesis),anddecreasedoxidationofFAinthe
mitochondriaduetoalcoholstoxicityonthemit

decreasedsubstrateforgluconeogenesis(pyruvatetolactateandnot
oxaloacetate);andpyruvatedoingonlylacticacidosisandnot
glycolysis

increasedacidproductioncompeteswithuricacidforexcretion

whatelsedecreasesproductionofoxaloacetatein
alcoholismotherthandecreasedpyruvate?

oxaloacetategetsshiftedtomalated/t

excessNADH

whatishepatocellularsteatosis?

whatliverenzymesareincreasedin
alcoholism?why?

whatdoesalcoholdotothe
P450system?

hepaticfattychange

AST(mitdamage),andGGT(d/tSERhyperplasiad/t
inductionofp450generation)

inductioninchronicalcoholism(increasestolerance);inhibition
inacutealcoholism

whyistheredecreasedimmunityinkwashiokorandmarasmus?

whatexplainsthefattychangein
kwashiokor?

abetalipoproteinemiaandincreasedG3Pproductionfromall
thecarbs!

whatistheswollenbellyfrominachildwithkwashiokor?

howdoesakwashiokor
patientpresent?

ascitesfromliverdamage

malnutrition,edema(hypoalbuminemia),anemialiver(fattychange),
diarrhead/tnobrushborderenzymes(noprotein!)

whatresultsinmarasmus?whatkindofmuscle
wasting?whatkindofmusclewastingdoyouseein
kwashiokor?

whatcausesliverdamagein
kwashiokor?

lossofsubcutaneousfat,tissueandmuscle
wasting(somaticmuscle);visceral,not
somatic

T/Fkwashiokorpatientsarecaloriedeficient

whatmetabolicreactions
occurinthecytoplasm?

noprotein!

false!

increasedTGsynthesisfromincreasedGlycerol3Pand
abetalipoproteinemia

glycolysis,fattyacidsynthesis,HMPshunt,proteinsynthesis
(RER),steroidsynthesis(SER),glycogenolysis,glycogenesis

whichmetabolicreactionsrequireboththemitand

hemesynthesis,ureacycle,

whatisthedifferencebetweenakinaseand
aphophorylase?

bothaddphosphate;phosphorylaseusesnoATPand
inorganicphosphate

whatistheratedeterminingenzymeinglycolysis?

phosphofructokinase1

whatistheratedeterminingenzymeingluconeogenesis?

fructose1,6bisphosphatase

whatistheratedeterminingenzymeinTCAcycle?

isocitratedehydrogenase

whatistheratedeterminingenzymeinglycogensynthesis?

whatistheratedeterminingenzymeinglycogenolysis?

whatistheratedeterminingenzymeinHMPshunt?

whatistheratedeterminingenzymeinureacycle?

glycogenphosphorylase

glucose6phosphatedehydrogenase

whatistheratedeterminingenzymeindenovopyrimidine
synthesis?

whatistheratedeterminingenzymeindenovopurine
synthesis?

glycogensynthase

carbomylphosphatesynthetaseII

glutaminePRPPamidotransferase

carbomylphosphatesynthetaseI

whatistheratedeterminingenzymeinfattyacidsynthesis?

AcetylCoAcarboxylase

whatistheratedeterminingenzymeinfattyacidoxidation?

carnitineacyltransferaseI

whatistheratedeterminingenzymeinsteroidsynthesis?
cholesterolsynthesis?

HMGCoAsynthetase;HMGCoA
reductase

whatistheratedeterminingenzymeingalactosemetabolism?

GALT

aldolaseB

whattypeofdiseaseisMELAS?whatisthe
clinicalpresentation?

mitencephalopathy,lacticacidosis,stroke
likeepisodes

whattypeofdiseaseisMERRF?whatistheclinicalpresentation?

whereistheredecreasedhydrogenconcentrationinthemit?
increased?

T/Ftheoutermitmembraneisimpermeabletoions

whattwoprocessesmaintainthe
plasmaglucoselevels?

ATPsynthase,ATPADP
translocase,ETC

32or30;malateaspartateshuttleor
glycerol3phosphateshuttle;heart/liverand
muscle

whatcarriesactivatedPhosphategroups?

whatcarrieselectrons(asenergy)?

ATP

NADH,NADPH,FADH2

coenzymeAandlipoamides

whatcarriesCO2asenergy?

whatcarries1carbonunitsasenergy?

succinatehydrogenase;
matrix

1)glycogenolysis(liveronlycontributestoplasma,muscle
keepsitforitself)2)gluconeogenesis

howmanyATPdoesaerobicmetabolismofglucose
produce?dependingonwhat?whattissuesuse
which?

whatcarriesAcyl(asenergy)?

matrix;intermembranespace

false!theinnermembraneis

whichTCAcycleenzymeisfoundintheinnermitmembrane?
wherearetherestfound?

otherthansuccinatedehydrogenasewhatotherenzymesare
foundintheinnermitmembrane?

myoclonusepilepsy

biotin

tetrahydrofolates

whatcarriesmethylgroupsasenergy?

SAM

whatcarriesaldehydesasenergry?

TPP(thiaminepyrophosphate)

T/FNAD+isusedinanabolicprocessesand
NADHisusedincatabolicprocesses

False!NAD+isusedincatabolicprocessesand
NADPHisusedinanabolicprocesses

GiveexamplesoftheanabolicprocessesthatNADPH
participatesin.FromwhatprocessisNADPHproduced?

Otherthananabolicprocesseswhatelseis
NADPHusedfor?

whatdohexokinaseandglucokinasedo?
usingwhat?whatpathwayisthisin?

T/Fglucokinaseis
ubiquitous

steroid,cholesterolFA
synthesis;HMPshunt

respiratoryburst,P450,andglutathione
reductase

phosphorylationofglucosetoyieldglucose6
phosphate;ATP;firststepofglycolysis

FALSO!hexokinaseis;glucokinaseonlyfoundinliverandbetacells
ofpancreas

T/Fhexokinasehasalowaffinityand
lowVmax

FALSO!hexokinasehasaHIGHaffinity(lowKm)and
lowVmax

T/Fglucokinasehasahighaffinityand
highVmx

falso!glucokinasehasaLOWaffinity(highKm)and
highVmax

ishexokinaseorglucokinaseactivatedbyinsulin?

T/Fhexokinaseandglucokinaseareinhibited
byexcessglucose6phosphate

T/Fglucokinaseisaglutton

whatisthepointofhavinga

glucokinase

falso!onlyhexokinase,glucokinasehasno
feedbackimhibition(itsaglutton)

TRUUUEEEEE.fattymcfatterson

sothatexcessglucosecanbestored(phosphorylated

whatisthenetproductionofglycolysisfromone
glucose?

whatistheratelimitingstepin
glycolysis?whatdoesitdo?who
activatesit?inhibitsit?

2NADH,2ATP,2pyruvate,2H+,2H20

Phosphofructokinase1;fructose1phosphatetofructose1,6
bisphosphate;enhancers:AMP,fructose2,6Bisphosphate;
downregulators:citrate,ATP

fromwhatispyruvateproducedinglycolysis?but
whatenzyme?whatenhancesthatenzyme?
downregulatesit?

whatensuresthatduringgluconeogenesisallthe
ATPwontbeusedinglycolysisasyoure
producingit?

phosphoenolpyruvate;pyruvatekinase;
fructose1,6BP;ATP,alanine

proteinKinaseAinhibitspyruvatekinasealso
ATPandalaninehavenegativeeffectson
pyruvatekinase

whatcontroloverpyruvatekinasedoesinsulinhave?

whatconvertspyruvatetoAcetylCoA?what
allostericallydownregulatesthisenzyme?

bywhatproductsare
gluconeogenesisandglycolysis
linkedsothattheyarenotboth
occurirngatthesametime?

T/Fgalactoseisthe
fastestsugar
metabolized.explain.

Pyruvatedehydrogenase;ATP,
NADH,acetylCoA

ATPisanallostericinhibitoronbothphosphofructokinase
andpyruvatekinaseandpyruvatehydrogenase;andfructose
2,6BPwhichisanallostericenhanceron
phosphofructokinaseisanallostericdownregulatoron
fructose1,6bisphophatase

whatisthefirststepinglycolysis?

howdoesfructoseenterglycolysis?
galactose?

itinducesitsaction!

phosphorylationofglucose

viafructose1Pbyfructokinaseintheliver;asglucose1by
beingGALTed;)

false!fructosebecausefructokinaseintheliverallowsittobypassthe
ratelimitingstepinglycolysis(PFK1),anditentersthroughadifferent
pathway(eitherthroughDHAPtoglycogenolysisortoglyeraldehyde
forglycolysis)

whatisfructose2,6phosphate?wheredoesit
comefrom?bywhatenzyme?

anallostericregulator;fructose1phosphate;

phosphofructokinase2

whatconvertsfructose2,6phosphatebacktofructose2
phosphate?

whattwoenzymesaretheapartofthesame
complex?whatdictateswhatformtheyare
in?andwhatdictatesthat?

whatdoesfructose2,6
bisphosphateregulate?

whatdoes
fructose6
phosphatedo,
dependingon
what?

fructosebisphosphatase2

fructosebisphosphatase2andPhosphofructokinase
2;phosphorylationbyproteinkinaseA;
inuslin/glucagonratio

positiveonphosphofructokinase1andnegativeonfructose
1,6bisphosphatase

thisdependsonifphosphofructokinase1isactiveandthisdependsonwhether
ornotfructose2.6bisphophateisactivatingitornotthiscanonlyoccurif
PFK2isonandnotFBPase2.thenfructose6phosphatewillparticipatein
glycolysis.OtherwiseFBPase2isonwhichwillresultinfructose6phosphate
goingtogluconeogensisbecausethereisnoF26BPtoactivatePFK1

T/Ffructose1,6bisphophateininvolvedin
allostericregulation

whenistherehighlevelsof
cAMP?whatdoesthis
resultin?

whenaretherelowlevels
ofcAMP?whatdoesthis
resultin?

FALSOfructose2,6bisphophateisinvolvedin
allostericregulation

whenthereisglucagon;proteinkinaseAactivationandthus
phosphorylationofthecomplexresultingintheactivationof
fructose2,6BPaseandnoPFK2

wherethereisinsulin;lowproteinkinaseAmeansdePhosphorylation
ofthecomplexwhichresultsinincreasedPFK2whichresultsinmore
fructose2,6bpandthusmorePFK1!!

T/FtheresultofdephosphorylationofPFK2isactivationofPFK1

YES!thinkitthrough...

T/Ftheglycerolphosphateshuttleresultsmoreenergythanthemalateaspartate
shuttle.
otherthantoglycolysis,wheredoesglucose6phosphate
getusedfor?iewhatotheractivatedcarrierstransmitits
energy?

FALSO

HMPshunt(toNADPH)and
glycogenolysis(asglucose1

phosphate)

otherthanglycogenesisandglycolysis,whatelseisFructoseusedfor?

whatisthepyruvatedehydrogenasecomplex
usedfor?whatisproducedinthatreaction?

conversionofpyruvatetoAcetylCoA;acetylcoA,
CO2,andNADH(oneofeachperpyruvate)

howmanyenzymesdoesthepyruvatedehydrogenasecomplex
have?andhowmanycofactors?whereisitlocated?towhat
enzymeisitsimilarto?

whatarethecofactorsrequiredfor
pyruvatedehydrogenase?

theproductsofFAoxidation(AcetylCoAandNADH)
phosphorylatepyruvatedeyhdrogenasewhichinactivates
it

acetylcoAandNADHinhibit
pyruvatedehyrogenase

Falseactivator!thislinksglycolysiswithmuscle
contraction

whatistheproductofalphaketoglutaratedehdrogenase?

whatdoesarsenicdo?howisthis
important?

3;5;mitmatrix;alpha
ketoglutaratedehydrogenase
complex

NADH,AcetylCoA,ATP;NAD+,ADPand
Ca2+

howarefaoxidationandgluconeogenesislinked,iewhat
compoundmakessurethattheyoccurtogetherandthatglycolysis
doesntoccur?

T/FCa2+isaninhibitoronpyruvate
deyhdrogenase

TPP(fromB1pyrophosphate),FAD(B2),NADH2
(B3),CoA(B5,panothenate),LipoicAcid

whatinhibitspyruvatedehydrogenase?what
activatesit?

inthefastingstate,whatensuresthat
thepyruvatedoesntgetusedforTCA
cycle?

mannose

succinylcoA

inhibitslipoicacid;lipoicacidisacofactorforpyruvate
dehydrogenase

whatarethesymptomsofarsenicpoisoning?

vomiting,ricewaterstools,garlicbreath

whatistheresultofpyruvate
dehydrogenasedeficiency?whatcausesit?

whataretworeasonswhy
pyruvatewouldbepushedto
lactateinanalcoholic?

backupofpyruvateandalanineandthuslactic
acidosisoccurs;canbed/talcoholism(vitB1def)

increasedNADHcauseslactateproductionandpyruvate
dehydrogenasedefcausesincreasedlactate(d/tthiaminedef
thatoftenoccurswithalcoholics)

whatarethefindingsinpyruvate
dehydrogenasedef?howdoyoutreat
it

neurologicdefects,myopathy,lacticacidosis;giving
ONLYketogenicnutrienthighfatcontentandaa(lysine
andleucine)

whichaaareonlyketogenic?

whatarethefourfates
ofpyruvate
metabolism?

pyruvatetoalanine(tocarryaminogroupstoliver),pyruvatetoOAAfor
gluconeogenesis(orreplenishforTCAcycle),acetylcoA(forTCAcycle
transitionorFAorchol.synthesis),ortheendofanaerobicglycolysis

whereisanaerobicglycolysisused
often?

whatisneededforglycolysistocontinue?
howisthisprovidedinaerobicglycolysis?
anaerobic?

whatistheendofanaerobic
glycolysis?

RBCs,renalmedulla,leukocytes,lens,testes,cornea

NAD+;throughETCyouproduceNAD+;through
productionofLactatefrompyruvateyoucreate
lactate

pyruvate!(notlactate,thatisgeneratejustinordertoproduce
moreNAD+)

whatdoestheTCAcycleproduce?

whatarethekeyintermediates
intheTCA?

lysineandleucine

3NADH,1FADH2,2CO2,1GTP=12ATP/acetylCoA

Citrate,Isocitrate,alphaketoglutarate,SuccinylCoA,Succinate,
Fumarate,Malate,Oxaloacetate

WheredoesNADH2enterETC?FADH2

ComplexI,ComplexII(lowerenergy)

Whatdoesthepassageofelectronsin
theETCresultin?

whatarethreewaysto
poisonOxphos?

formationofaprotongradientthatisthenusedtocreate
ATPthroughATPsynthase

electrontransportinhibitors,ATPaseinhibitors,Uncouplingagents
(increasepermeabilityofthemembranelossofhydrogengradient)

Whatarerotenon,CN,antimycinA,CO?

whatdoesoligomycin
do?

whatareuncoupling
agents?whatisthe
result?

blockATPsynthaseresultinginnoATPproductionandanincreased
protongradient

theyincreasethepermeabilityoftheinnermitmembraneresultingina
lossofgradientandlossofATPproductionBUTelectrontransport
continues!heatisgeneratedinstead

T/FO2consumptionstopswith
uncouplingagents

listthreeuncouplingagents

FALSO!becauseelectrontransportcontinues,O2consumption
(lastelectronacceptor)getsconsumedalso

2,4dinitrophenol,aspirin,andthermogenin(inbrownfat)

whataretheonlysubstratesfor
gluconeogenesis?andwhat
productsareconvertibletoit?

T/FacetylcoAisasubstrate
forgluconeogensis

blockElectrontransport

pyruvate(throughOXA);lactate,alanine,malate(andall
otherTCAintermediatesthroughOXA),glycerol,oddchain
fattyacidsyieldpropionylcoAwhichcanbeconvertedto
succinylcoAandenterasTCAcycle

FALSEWECANTUSEevenchainfattyacidsFORBLOOD
GLUCOSEORELSEWEWOULDALLBEANOREXIC

whatarethefourirreversibleenzymesof
gluconeogenesisandwhereinthecell
aretheyfound?whichisdeficientinvon
gierkesdisease?

pyruvatecarboxylase(pyruvatetoOXA)(mit),PEP
carboxykinase(OXAtoPEP)(cyt),Fructose1,6
bisphophatase(fructose1,6bisphosphatetofructose6
P)(cyt)andglucose6phosphatase(inER);glucose6
phosphatase

wheredoesgluconeogenesisoccur?

livermitandlivercytosol

otherthanAcetylcoAinhibitingpyruvatedehydrogenase
complex,howelseisitensuredthatgluconeogenesisisoccuring
duringFAoxidationandnotglycolysis?

AcetylcoAactivatedpyruvate
carboxylasein
gluconeogenesis

whatisapositiveallostericregulatoronpyruvatecarboxylase?for
whatreactionisthis?

acetylcoA;
gluconeogenesis

whattwosubstrates(otherthanpyruvate)doespyruvatecarboxylaserequire?

whatdoesPEPcarboxykinaserequire?andfor
whatreactionisthis?

whatregulatesFructose1,6
bisphosphatase?whatreactionisitfound
in?

citrateispositiveonit,AMPandfructose2,6
bisphosphateisnegativeonit;gluconeogenesis

kidneyandintestinalepithelium

whycantmuscleparticipateingluconeogenesiswhatkeyenzymedoes
itlack?

glucose6phosphatase

False!oddchainfattyacidsyieldoneproprionylcoAwhich
canentertheTCAcycleassuccinylcoA(andthentoOXA)

T/Fevenchainfattyacidscannotproducenewglucosesincetheyonlyyieldacetyl
coA

whatisonesteroidthatincreasesgluconeogenesis?whatelsedoes
cortisoldothatyieldshyperglycemia?

whatisthepurpose
ofHMPshunt?

ATPandbiotin

GTP:gluconeogenesisOXAto
phosphoenolpyruvate

otherthaninthekidney,whereelsearegluconeogenesis
enzymesfound?

T/Fallproductsoffatscannot
entergluconeogenesis.

TRUE

cortisol;inhibitsinsulin
action

toprovideNADPHsincethereisanabundanceofglucose6phosphate;
alsoyieldsribosefornucleotidesynthesisandglycolysisintermediates

howmanystagesarethereinHMP

2oxidativeandnonoxidative;cytoplasm(both);none

wheredoestheHMP(pentose
phosphatepathway)occurotherthan
intheliver?

lactatingmammaryglands,adrenalcortex(sitesoffatty
acidorsteroidsynthesis),RBCS(forglutathionereduction
forFRinjury)

whatenzymeisusedintheoxidativephaseofHMPshunt?
nonoxidative?

howdoeslactatecontributeto
gluconeogenesis?

G6PDdehydrogenase;
transketolases

getsconvertedtopyruvateviatheCoricycleinthe
liver

namethreeglucogenicAAandhowtheyenter
gluconeogenesis.throughwhatcycledotheyenter
gluconeogenesis?

howdoesglycerolfromtriacylglycerolsin
adiposetissueusedforgluconeogenesis?

alanine(throughpyruvate),aspartate
(throughOXA),glutamate(alpha
ketoglutarase);TCA

getsconvertedtoglycerol3Pwhichisthen
convertedtoDHAPwhichthencanenter
glycolyis

T/Fglycogenmakesupthebackwallof
triacylglycerolinfat(TG)

false!glycerol;glycogeniswhatglucoseis
storedas

Whatisthefirststepintherespiratoryburst/oxidativeburst?inwhat
diseaseisthisdeficient?

inwhatcellsdoestheRespiratoryburstoccur?
whereisNADPHlocatedinthecell?whatisthis
reactionimportantfor?

whatarethefirstthreeenzymesinthefirstthree
stepsinrespiratoryburst?wheredotheyoccur?
whatdotheyresultin?whatdoesitdo?

NADPHoxidase;CGD

Neutrophilsandmonocytes;membranebound;
immuneresponsebygeneratingReactive
oxygenspecies

whatisthisreactiondependenton?

whatarethelastthree
stepsoftherespiratory
burst?whydotheyneedto

oxygen!

NADPHoxidase,superoxidedismutase,
myeloperoxidase;inthephagolysosome;
HOCl(bleach);kills!

glutathioneperoxidase(catalase)reducesH202toH20with
glutathione;oxidizedglutathionegetsreducedbyglutathione

reductasewithNADPH;NAP+isregeneratedtoNADPHwith
Glucose6phosphatedehydrogenase;becauseH2O2fromtheresp
burtsinthephagolysosomesomegetsleakedintocell;inthecytosol

occur?wheredothey
occur?

howdoWBCsofCGD
patientsdosomedamage?

theyusetheH2O2producedbysomebacteriatoproducebleach
withmyeloperoxidasewhichtheystillhave

WhatkindofbugsareCGDpatients
screwedwith?whydotheycallit
chronicgranulamotousdisease?

catalasepositivebecausetheyreducetheirH2O2and
WBCscantusethattomakebleach;becausethewaythey
protectisbybuildingawallaroundtheorganismswith
granulomas

whataresomecatalasepositivebugs?

s.aureus,Aspergillus

otherthantheH2O2producedfromtherespburstin
infections,whatotherthingsdoesG6PDreproduceNADPH
forglutathionereductionfor?whatcellsareparticularly
vulnerabletotheseoxidizingagents?

whathappensinRBCsaftertheir
membranesgetoxidized?

whatisthepresentationof
G6PDdeficiency?

hemolyticanemiawithabloodsmearwithheinzbodiesandbite
cells(phagocyticremovalofheinzbodies)

whatarethetitlesofthetwotypesoffructose
disorders?whichismoreserious>

T/Ffructosedisordersareworsethangalactosedisorders.

whatisthe

sulfonamides,primaquine,
dapsone,antiTBdrugs,fava
beans,LDL;RBCs

hemolyticanemiaduetoheinzbodieswhichareoxidized
hemoglobinprecipitatingwithintheRBCs

whatisthemostcommonhumanenzymedeficiency?why?
whatisthemodeofinheritanceofthisdeficiency?

T/Fgalactosecannotgetinsidecells

G6PD;providesmalarial
resistance;Xlinkedrecessive

essentialfructosuriaandfructoseintolerance;
fructoseintolerance

false

false;fructosecantgetinsidecells

fructosegetsphosphorylatedintofructose1Pbyfructokinaseandthen

whatenzymeisdeficientinEssentialfructosuria?
whatarethesymptoms?

whatenzymeisdeficientinFructose
intolerance?whatistheresultofthe
enzymedef?whatarethesymptoms?

howdoyoutreatfructose
intolerance?

AldolaseB;Fructose1phosphateaccumulateswhichuses
upPhosphateandtheresultisinhibitionof
gluconeogenesiswithoutphosphate;hypoglycemia,
jaundice,cirrhosis,vomitting

decreaseintakeoffructoseandsucrose(fructoseand
glucose)

whatarethetwodisordersofgalactose
metabolism?whichisworse?

whatisthe
metabolismof
galactoselike?

fructokinase;benignfructoseintheblood
andurine

galactokinasedeficiencyandclassicgalactosemia;
classicgalactosemia

galactosegetsconvertedtogalactose1Phosphatebygalactokinasewhichthen
getsturnedintoglucose1phosphatebyGalactose1UridylTransferase
(GALT)whichalsoproducesUDPGalwhichisusedforlactoseproductionin
thebreast;thefateofglucose1dependsonfasting(glycolysis)orfedstate
(glycogenesis)

whattissuescontainaldosereductase?what
doesthisenzymedotowhat?

lensandneuraltissue;convertsgalactoseto
osmoticallyactivegalactitol

wheredoesamajorityofgalactoseinourdietcomefrom?

whatcausescataractsinagalactose
metabolismdeficiency?

whatenzymeisdeficientin
galactokinasedeficiency?whatare
thesymptoms?

whatenzymeisdeficientin
classicgalactosemia?
symptoms?treatment?

lactose(glucoseandgalactose)

aldosereductaseinthelensconvertsbuiltupgalactoseto
galactitolwhichisosmoticallyactive

galactokinase;galactoseappearsinbloodandurine,
sometimesinfantilecataractsmaypresentasfailuretotrack
objectsorlackofasocialsmile

galactose1phosphateuridyltransferase(GALT)resultsinan
accumulationoftoxicsubstances;failuretothrive,jaundice,
hepatomegaly,infantilecataracts,mentalretardation;exclude
galactoseandlactosefromdiet

whydoesgalactosedefpresentinnewborns>

whatisanotherwaytotrapglucoseincells
butnotbyphosphorylatingit?what
enzymesareused?

inwhattissuescansorbitol
accumulate?why?

byturningitintofructose,butsorbitolfirst;glucose
tosorbitolviaaldosereductaseandsorbitolto
fructosesorbitaldehydrogenase

schwanncells,lens,retina,kidneysbecausetheyonlyhavealdose
reductaseandnosorbitaldehydrogenase

whatisosmoticdamageandwhatmolecules
causeit?givesomeexamplesofosmotic
damageinwhatdiseasestatedoyousee
these?

T/Fosmoticdamagecan
notoccurintheblood

lactoseinmothersmilkcontainsgalactose

whenwaterispulledin;polyols(sugaralcohols);
cataracts,retinopathy,peripheralneuropathyall
seeninchronichyperglycemiaindiabetes

falsehighbloodlevelsoffructose,galactose,glucosecanresultin
conversiontoosmoticallyactivealcoholformsbyaldosereductase!

whatistheresultoflactase
deficiency?inwhatnationalitiesisit
seenthemost?

lackoflactase(brushborderenzyme)socannotbreak
downlactose(adissaccharideofgalactoseandglucose);
AfricanAmericansandAsians

whatarethesymptomsinvolvedinlactasedeficiency?

whatisuniqueaboutthediarrheain
lactasedeficiency?

osmoticdiarrhea,bloating,cramps

bacteriaproducelactateacidandgasesfromlactoseand
produceacidicdiarrheaandbloating

otherthanbeinghereditary,whataretwootherways
youcanhavealackofbrushborderenzymes?

followinggastroenteritisandkwashiokor
(proteindeficiency)

T/FaminoacidsarefoundinbothLandDforms

whataretheketogenicaminoacids?

falseonlyLform

leucineandlysine

whataretheglucogenicaminoacids?

met,arg,val,his

whataretheglucogenicandketogenicaminoacids?

Ile,Phe,Thr,Trp

whichaminoacidsareacidic?aretheynegativelyorpositivelycharged
atbodypH?

whichaminoacidsarebasic?aretheynegativelyor
positivelychargedatbodypH?

arg,lys,his;positiveexceptHishasno
chargeatbodypH

whichaminoacidisthemostbasic?

whichaminoacidsmakesuphistones?

arg

lysandarg

whichaminoacidsarerequiredduringperiodsofgrowth?

whathappensinan
aminotransferase
reaction

aspandglu;negatively

argandhis

anaminotransferaseenzymetransfersanaminogroupfromanamino
acidontoalphaketoglutaratemakingitaglutamate(tojointheurea
cycleorexcretedinthekidney)andtheaminoacidthatlosttheamino
groupisthenturnedintoaglycolyticintermediate(pyruvateor
oxaloacetate!)

howarenonessentialaminoacidsmade?

whatarethetwomostcommonaminotransferase
andwhataminoacidsdotheyturntowhat
glycolyticintermediates?

alanineaminotransferase(ALT)andaspartate
aminotransferase(AST);alaninetopyruvate
andaspartatetooxaxloacetate

wheredoaminotransferasereactionsoccur?andwhatvitamincofactordo
theyrequire?
howisexcessnitrogentakencare
of?

...

inhepatocytes;B6

excretedfromthekidneyintheformofureainhepatocytes

asBUN(bloodureanitrogen)

howisureameasured

whicharethemitochondrial
reactionsoftheureacycle?

carbomylphosphatesynthetaseIturnsNacetylglutamateinto
carbomylphosphateandthenornithinetranscarbomyalseturns
carbomylphosphateintocitrulline(usingornithine)

whichistheratelimitingstepoftheureacycle?

whattwocompoundsprovidethetwoN'sofurea?what
elseisinureaotherthantwoNH2's?

aftercitrullineis
producedandinthe
cytoplasmwhathappens
nextintheureacycle?

glutamateandaspartate;CO2
providesanketone

ornithinetranscarbomyalse;orotic
aciduria;Xlinkedrecessive;
autosomalrecessive

asalanine;aminoacidsdonatetheiraminogrouptoalphaketoglutarate
makingitglutamateandtheaathenbecomesanalphaketoacid(joins
TCAcycle);glutamatethenconvertspyruvatetoalaninebydonatingits
aminogrouptopyruvate(withALT);alaninethentravelsthroughthe
bloodandinthelivergetsreconvertedbacktopyruvate
(gluconeogenesis)whilegivingitsaminogrouptoanalpha
ketoglutaratewhichmakesglutamatewhichthenjoinstheureacycle

whatistheprimarysourceofnitrogenintheureacycle

T/Fallglutamategoestolivertobeturned
intourea

howcanhyperammoniabe
hereditary?acquired?

citrullineiscombinedwithaspartatetomakearginosuccinatewhichis
thensplitintofumarate(whichleavestoenterTCA)andarginine.
ArginineisthenconvertedtoOrnithine(viaarginase)givingoffUrea
whichthengoestothekidneyandornithinereenterstheureacycle
(combinedwithcarbomylphosphatetomakecitrulline)

whatisthemostcommondisorderoftheureacycle?whatis
theresult?whatisitsmodeofinheritance?whatisthemode
ofinheritanceoftheotherureacycleenzymedef?

howisthenitrogenof
aminoacidstransferred
totheliverfortheurea
cycle?explainthis
process.

carbomylphoshpatesynthetaseI

glutamate

false;goestokidneyandammoniaisusedasabuffer
inurine

ureacycleenzymedeficiency(mc:ornithine
transcarbamoylase);liverdiseasealcoholiccirrhosisandreye's

whatdoestheexcessNH4+inhyperammoniaeatup?
whatistheresult?

whatisthetreatment
ofhyperammonia?

alphaketoglutarate;noTCAcycle
intermediates

limitproteinindiet;givebenzoateorphenylbutyratewhichbindamino
acidsandleadtoexcretion,lactulose(hydrogenionbindstoammoniato
makeexcretableammonium)andneomycin(tokillbacteriathatrelease
ammoniafromaa)

whataretheclinicalsymptoms
ofhyperammonemia?

tremor(asterixis),slurringofspeech,decreasetemperature,
somnolence,vomitting,cerebraledema,blurringofvision,
neuralfocaldeficits

whatistheresultofarginasedeficiency?

whatarethelabfindingsseein
hyperammonemia?

whatarethefindingsinornthinie
transcarbamoylase?

choreaandspasticparesis

increasedbloodlevelsofammonia,decreased
BUN

oroticacidinbloodandurine(frompyrimidinesynthesis),
decreasedBUN,andsymptomsofhyperammonemia

whataadoestyrosinecomefrom?

phenylaline

fromwhataadoesdopacomefrom?

whatconvertsdopaintomelanin?

tyrosinase

fromwhataadoesthyroxinecomefrom?

fromwhataadothecatecholaminescomefrom?andwhataadoesthataa
comefrom?

whataadoesmelanincomefrom?melatonin?

tyrosine

fromwhataadoesniacincomefrom?withwhatvitamincofactor?

tyrosine

tyrosine;phenylaline

tyrosine;tryptophan

tryptophan;B6

fromwhataadoNAD+andNADP+comefrom?

tryptophan

fromwhataadoesserotonincomefrom?whatNTisserotonina
precursorfor?

tryptophan;melatonin

fromwhataadoeshistaminecomefrom?withwhatvitamincofactorshelp?

histidine;B6

fromwhataadoesporphyrincomefrom?whatisporphyrintheprecursorof?

glycine;heme

forwhatthreeenzymesisarginineaprecursorfor?

NitricOxide,creatinine,Urea

whataa(otherthanglutamate)isaprecursorforurea?

whataaistheprecursorforNO?

arginine

arginine

whataaistheprecursorforcreatinine?

arginne

fromwhataadoesglutathionecomefrom?

glutamate

fromwhataadoesGABAcomefrom?withwhat
enzyme?andwhatvitcofactor?whatdoesGABAstand
for?

whatarethethreebranchedaa?

whatenzymeisdeficientinmaplesyrup
disease?whatdoesitresultin?

isoleucine,valine,leucine

branchedchainalphaketoaciddehydrogenase;
inabilitytobreakdownbranchedchainaa

whataaistheultimateprecursorofcatecholaminesynthesis?
whatenzymeconvertsphenylalaninetotyrosine?
whatcofactorisused?

glutamate;glutamatedecarboxylase;
B6;gammaaminobutyrate

phenylalanine

phenylalaninehydroxylase;

tetrahydrobiopterinfactor

whatconvertstyrosinetoDOPA?what
cofactorisused?

tyrosinehydroxylase;tetrahydrobiopterinfactor;
NADP+

whatisdihydroxyphenylalanine?

T/FtyrosinaseconvertstyrosinetoDOPA

DOPA

false;tyrosinehydroxylase

whichcatecholaminehasinhibitoryactionagainstacetylcholine?

whatconvertsdihydrobioterinbacktotetrahydrobioterin
factor?whatenergycarrierisused?inwhatreactionsis
thiscofactorused?

dihydropterinreductase;NADP+;
phenylalaninetotyrosineandtyrosineto
DOP

whatconvertsdopatodopamine?usingwhatcofactor?

wheredoescatecholaminesynthesistakeplace?

dopadecarboxylase;B6

neuraltissueandadrenalmedulla

whatconvertsdopaminetonorepinephrine?usingwhat
cofactor?

T/Fvitcisneededincatecholaminesynthesis

dopaminebetahydroxylase;vitC

true!dopaminetonorepinephrine

whatnegativelyregulatesdopaminetonorepinephrine?

whatenzymeconvertsnorepinephrinetoepinephrine?
usingwhatcofactor?

carbidopa

phenylethanolamineNmethyltransferase;
SAM

whatisphenylethanolamineNmethyltransferaselocated?

T/FphenylethanolamineNmethylisonlylocatedinthe

dopamine

adrenalmedulla

true(epinephrineonlymadeinthe

whatisthedegradationproductofNorepinephrine?

whatisthedegradationproductofdopamine?

whatisthedegradationproductofepinephrine?

whattwodef.mostcommonlycause
phenylketonuria?

phenylalaninehydroxylaseortetrahydrobiopterin
factor

whatexplainstheexcessphenylketonesintheurinein
phenylketonuria?

tyrosine

phenylalaninebuildup

alsohavefairskin,blondhair,blueeyes,
eczema

mentalandgrowthretardation;seizures,fairskin,eczema,musty
bodyodor;neurotoxicketonesandacidsstimulatevomittingcan
leadtopyloricstenosis

whatisthetreatmentforphenylketonuria?

whatis
MaternalPKU?

COMTandMAO

whataabecomesessentialwithphenylketonuria?

whatarethefindingswith
phenylketonuria?

HVA

metanephrine

whattwoenzymesareininvolvedincatecholaminedegradation?

giveanexampleofhowphenylketonuriais
pleiotropic.

VMA

decreasedphenylalanine,increasetyrosine

lackofproperdietarytherapyduringpregnancyresultingininfant's
microcephaly,mentalretardation,growthretardationandcongenitalheart
defects

whattypeofaaisphenylalanine?whatimplicationsdoesthishaveon
thepresentationofphenylketonuria?
whatarephenylacetate,phenylactate,and
phenylpyruvate?

aromatic;mustybody
odor

phenylketonesthatbuildupin

phenylketonuria

whatisthemodeofinheritanceofphenyketonuria?

whenisphenylketonuria
screened?why?

23daysafterbirthbecausematernalenzymeduringfetallike
mightstillbethere

whatdoesadeficiencyinhomogentisticacidoxidaseresultin?whatis
thisalsoknownas?
whatenzymeisinvolvedinthedegradationoftyrosineto
fumarate?

namethreecausesofalbinism.what
doesalbinismincreasedriskof?

T/FAlbinismis
AR

alkaptonuria;ochronosis

homogentisticacidoxidase

whatbuildsupintheurineinhomogentisticacidoxidasedeficiency?
whatdoesthisresultin?

whatarethefindingsin
alkaptonuria?whydothey
occur?

AR

urineturnsblackon
standing

darkconnectivetissue,brownpigmentedsclera,debilitating
athralgias;homogentisticacidbuildsupandcanbetoxicto
cartilage

tyrosinasedeficiency;defectivetyrosinetransporters;lack
ofmigrationofneuralcrestcells;skincancer

false;onlythetyrosinaseoneisalbinismhasvariableheritabilitybecauseof
locusheterogeneity

T/FOcularalbinismisAR

falseXlinkedrecessive

T/Fhomocysteinegetsturnedintomethioninebyhomocysteinetransferase

otherthanbeingreturnedtomethionine,whatelseis
thefateofhomocysteine?throughwhatenzyme?with
whatcofactor?
whatdoesSAMturnintoafteritlosesa
methyl?howdoesitgetturnedbacktoSAM?

true

cystathionine(whichthengoesto
cysteine)viacystathioninereductase;B6

homocysteine;homocysteinemethyltransferase

withB12andtetrahydrofolate

whatthreeenzymedef.cancause
homocysteinura?whatistheirmodeof
inheritance?

homocysteinemethyltransferase,cystathioninereductase,
decreasedaffinityofcystathioninereductasefor
pyridoxalphosphate;AR

whataabecomesessentialwithhomocystinuria?phenylalaninuria?

howdoyoutreathomocystinuriaduetodecreasedaffinity
ofcystathioninereductasetoB6?

whatarethefindingsof
homocystinuria?

proprionylcoA;through
methylmalonylcoA;B12

hereditarydefectofrenaltubularaminoacidtransporterforcysteine,
ornithine,lysineandarginineinthePCTofkidnyes

whatisthepresentationforcystinuria?

whatisthetreatmentforcystinuria?

T/Fcysteineisadimer

bygivinghighamountsofpyridoxal
phosphateinthediet

homocystinuria(homocysteinedamagesendothelial
cells);alkaptonuria(tyrosinedegradation);
homocystinuria

whatistheproductofoddchainfattyacidsynthesis?how
doesitreturntoTCAcycle?whatcofactorisrequired?

cysteine;tyrosine

increasedhomocysteineintheurine,mentalretardation,
osteoporosis,marfanoidhabitus,andatherosclerosis

whataadegradationdisorderresultsin
atherosclerosis?debiltatingarthralgias?
osteoporosis?

whatiscystinuria
dueto?

cystinekidneystones(cystinestaghorncalculi)

acetazolamidetoalkalinizetheurine

trueoftwodimersconnectedbydisulfidebond

whatenzymeisnecessaryforbranchedaa
degradation?whatcofactor?whatarethebranched
aa?

brancheaaalphaketoaciddehydrogenase;
thiamine;Isoleucine,leucine,valine

alphaketoacidsintheblood;alphaketoacids

whatenzymeisdeficientinmaplesyrupurine
disease?

whatarethefindingsinmaplesyrupdisease?
whatshouldbetriedtobegivenastreatment?

whatishartnupdisease?whatisthemode
ofinheritance?

branchedalphaketoaciddehydrogenase

CNSdefects,mentalretardation,death,feeding
difficulties,vomiting,hypoglycemia;thiamine

defectiveneutralaminoacidtransporteronrenaland
intestinalcells;AR

whataaismostaccumulatedinhartnupdisease?whatvitaminbecomes
deficient?

whatisthemainclinicalpresentationofhartnupdisease?

whatisthemainhormonalregulationofglycogen?

T/Fthemainhormonalregulationofglycogenisglucagon
andinsulin

whereisglycogenmainlyfound?

whatisthemainuseof
glycogen?

tryptophan;niacin

pellagra

glucagon,insulinandepinephrine

falseglucagon,insulinand
epinephrine

liverandskeletalmuscle

reservesupplyofglucoseinthefastingstatelivergivestotheblood
andskeletalmuscleusesforitself

T/FincreasedlevelsofcAMPresultsinglycogenolysis

true

T/Fglucagonworksontheliverandmuscletoactivateglycogenolysisby
activatingadenylylcyclase

falsejustliver

T/Fepinephrineworksonlivertoactivateglycogenolysisbyactivating
adenylylcyclase

falseliverandmuscle

T/Fphosphorylatedglycogenphosphorylaseisinactive

whatisthefirstenzymestepinglycogenolysis?

false

glycogenphosphorylase;phosphorylation

howdoesglucagonactivate
glycogenolysis?whatregulator
doessimilar?

byactivatingadenylylcylasewhichincreasescAMPwhich
increaseslevelsofproteinkinaseAwhichactivatesglycogen
phosphorylasekinase;epinephrine

howismuscleactivityandglycogenolysis
coupledinmuscle?

T/Finuslindownregulates
glycogenolysisbyinactivating
cAMP

calciumandcalmodulinactivateglycogen
phosphorylasekinase

falseinsulinreceptorisnotcAMPsecondreceptorbutrater
tyrosinekinasewhichdimerizesandactivatesaprotein
phosphatasewhichdephosphorylatesglycogenphosphorylase

howdoesinsulinbothdeactivate
glycogenolysisandactivates
glycogenesisatsametime?

byactivatingproteinphosphatasewhichboth
dephosphorylatesglycogenphosphorylase(deactivating)
anddephosphorylatesglycogensynthase(activatingit)

T/Finsulinactsonthelivertodeactivate
glycogenolysis

falsebothliverandmuscle(unlike
glucagon)

T/Fglycogenbrancheshave1,4bondsandlinkageshave1,6bonds

T/Fglycogenolysisinskeletalmusclemaintainsbloodsugar

whatkindoflinkagesdoesglycogensynthasemake?
branchingenzyme?

whydoesbranchingoccurin
glycogenesis?

falseopposite

false;inhepatocytes

alpha1,4linkages;alpha1,6
linkages

branchingincreasestherateofsynthesisandbreakdownof
glycogenbyhavingmoresitestoaddontoandbreakoff

afterglucose6Pisconvertedtoglucose1P,
whatisthenextstepinglycogenesis?then
what?

conversiontoUDPglucoseviaUDPglucose
pyrophosphorylase;glycogensynthase(rate
limitingstep)

whatisthemainformofglycogenolysisafterglycogen
phosphorylase?whathappensinsteadinsomelysosomes?does
thisprocessuseglycogenphosphorylase?inwhatdiseaseisthis
enzymedeficient?

debranchingenzyme;alpha1,
4glucosidase;no!;Pompes

disease

whatdoesglycogenphosphorylaseinglycogenolysiscreate?

whataretheglycogenstoragedisease?
whattheyresultin?

abnormalglycogenmetabolismresultsinaccumulation
ofglycogenincells

T/FalltheglycogenstoragediseasesareAR

whatisthefateofglucosefromglycogenolysis?

howdohepatocytesreleaseglucose6Pfromglycogenolysisinto
theblood?inwhatdiseaseisthisenzymedeficient?

whatisthedeficientenzymeinVon
Gierkesdisease?whatarethefindings?

whatistheorderoftypesof
glycogenstoragedisease?

WhatarethefindingsforPompe's
disease?whatisthedeficient
enzyme?

findingsforMcArdle's
disease?deficient
enzyme?

true

glucosekinasetoglucose6P

withglucose6phosphatase;
VonGierkesdisease

severefastinghypoglycemia,veryhighlevelsglycogen
inliver,highbloodlactate,hepatomegaly

VeryPoorCarbMetabolism(VonGierkeType1,Pompes
TypeII,Cori'sTypeIII,McArdlesdiseaseTypeIV)

FindingsforCori'sdisease?
deficientenzyme?

limitdextrans

cardiomegalyandsystemicfindingsleadingtoearlydeath;
lysosomalalpha1,4glucosidase(acidmaltase)

milderformoftype1withnormalbloodlactatelevels;
debranchingenzyme(alpha1,6glucosidase)

highglycogeninmuscle,butcannotbreakitdown,leadingtopainful
musclecramps,myoglobinuriawithstrenuousexercise,noincreasein
lacticacidafterexercise;skeletalmuscleglycogenphophorylase

whichglycogenstoragediseaseresultsinhepatomegaly?

whichglycogenstoragediseasecauseshypertriglyceridemia?

vongierkes

vongierkes

whichglycogenstoragediseaseresultscardiomegaly?

T/Fcorisglycogenstoragediseasehasnormallactatelevels

whichglycogenstoragediseaseresultsinnolacticacidafterexercise?

whatcausesthelysosomal
storagediseases?

pompes

true

mcardlesdisease

deficienciesinoneofthemanylysosomalenzymesresultsin
accumulationofabnormalmetabolicproducts

whichtwolysosomalstoragediseaseareXRandnot
AR?

FabrysDiseaseandHuntersSyndrome

whatisthedeficientenzymeinFabry'sdisease?
accumulatedSubstrate?

alphagalactosidaseA;ceramide
trihexoside

whatisthedeficientenzymeinGauchersdisease?
accumulatedSubstrate?

Betaglucocerebrosidase;
glucocerebroside

whatisthedeficientenzymeinNiemannPickdisease?
accumulatedSubstrate?

whatisthedeficientenzymeinTaySachsdisease?
accumulatedSubstrate?

whatisthedeficientenzymeinKrabbesdisease?
accumulatedSubstrate?

whatisthedeficientenzymeinMetachromaticleukodystrophy
disease?accumulatedSubstrate?

whatisthefindingsin
Fabrysdisease?
whichisthemostcommon
lysosomalstoragedisease?

Sphingomyelinase;sphingomyelin

HexosaminidaseA;GM2
ganglioside

Galactocerebrosidase;
Galactocerebroside

arylsulfataseA;cerebroside
sulfate

peripheralneuropathiesofhands/feet;angiokeratomasbetween
umbilicusandknees;cardiovascular/renaldisease

Gauchers;hepatosplenomegaly,asepticnecrosisofthefemur,

bonecrises,Gaucherscellscrumpledtissuepaperlooking
(fibrillarappearingmacrophagesinliver,spleen,b.m.)

whatareitsfindings?

whatarethefindingsin
NiemannPickdisease?

whatarethefindings
inTaySachsdisease?

progressiveneurodegeneration(spasticityd/tUMNdisease),
hepatosplenomegaly,cherryredspotonmacula,foamcells

progressiveneurodegeneration(spasticityd/tUMNdisease),NO
hepatosplenomegaly,cherryredspotonmacula,lysosomeswithonion
skin,muscleweakness

whatarethefindingsinKrabbes
disease?

peripheralneuropathy,developmentaldelay,opticatrophy,
globoidcells

whatarethefindingsinMetachromatic
leukodystrophy?

centralandperipheraldemyelinationwithataxia
anddementia

whichtwolysosomalstoragediseasesfindingsarevery
similar?howdoyoudifferentiatethem?

whatarethemucopolysaccharidoses?which
oneisXR?AR?whataccumulatesinthem?

NiemannpickandTaySachs;
hepatosplenomegalyornot

HurlersSyndromes;Iduronatesulfatase
deficiency;alphaLiduronidasedef;heparan
sulfate,dermatansulfate

whichHurlersismilderandpresentswithnocornealclouding?

whatarethefindingsin
ARHurlers?

XRiduronatesulfatase

developmentaldelay,gargoylism,airwayobstruction(d/tshortneck),
cornealclouding,CAD,hepatosplenomegaly

whatlysosomalstoragediseasesareseenmorein
AshkenaziJew?

whyistherehepatosplenomegalyinmanyofthe
lysosomalstoragediseases?

taySachs,NiemannPicks,Gauchers

becausecellsofmononuclearphagocyticare
esprichinlysosomes

whichlysosomalstoragediseaseshaveenzymesthataredefincells
thatareneuronalmostly?

neimannpickandtay

sachs

inFabrys,whataccumulatesintissuebecausefibroblastsexpressthedef
enzymehighly?

ceramidetrihexoside

whatisthefirststepinfattyacidsynthesis?
wheredoesitoccur?wheredoes
triacylglycerolsynthesisoccur?

citrateshuttleintheinnermitochondrial
membranetransportsacetylcoAintothe
cytoplasm;liver;liverandadiposetissue

whatistheratelimitingstepinfattyacid
synthesis?whatfourthingsinhibitthisrate
limitingstep?whatstimulatesit?

acetylcoAcarboxylaseconvertingAcetylcoA
intomalonylcoA;increasedAMP,glucagon,
epinephrine,palmitate;citrate

wheredoessynthesisoflongerchainfattyacidsoccur?what
doesitrequire?

whatispalmitate?saturatedorunsaturated?
whatdoesthatmeat?

false!NADPHreductivebiosynthesis!

whatcofactordoesacetylcoAcarboxylase
require?whatdoesitconvert?

T/Fcarnitineshuttleisusedtotransportacetyl
coAoutofthemitochondriaforfattyacid
synthesis
whatdoeslipolysisgenerate?and
whattworegulatorsactivate
lipases?

whatisthefateofthetwo
productsoflipolysis?

SERandmitochondria;NADPH

a16CFA!theproductinFAsynthesis;
saturated;nodoublebonds

T/FfattyacidsynthaserequiresNADP+

biotin;ratelimitingstepinfAsynthesisacetyl
coAintomalonylcoA

false!citrateshuttle;carnitineshuttletakes
acylcoAintothemitforfattyaciddegradation

triacylglycerolsinadiposetissuebrokendownintoglycerol
andfreefattyacids;growthhormoneandepinephrine

glycerolgoestoliverforgluconeogenesis;freefattyacidsbindto
albumininbloodandtraveltocellstobeusedasforenergy

T/FFattyacidcoAsynthetaseisusedinFAsynthesis

falseFAdegradation

FAcoAsynthetasecombinescoAwithFAtomake

whatisrequiredtogetAcylcoAinFAdegradationintothemitochondriafor
betaoxidationtoacetylcoAgroups?

whatiscarnitinedeficiency
andwhatdoesitresultin?

carnitineshuttle

inabilitytotransportLCFAintomitochondriaresultsintheirbuild
upandcausesweakness,hypotoniaANDHYPOKETOTIC
HYPOGLYCEMIA

whatisdeficientinsomeonewithhereditaryhypoketotichypoglycemia?

whydoesfadegradationoccurinthe
mitochondria?whatdoesFAdegradationproduce?
whatisthefateofthisproduct?

whatisacylcoAdehydrogenaseusedin?
whatdoesadeficiencyinthisenzyme
produce?

walkthroughtheprocessthat
adiposetissuemakes
triacylglycerol.

whatisthemainsourceofFA?
whereelsecantheybereleased
from?

T/Ftheliverusesketone
bodiesforenergy

carnitineshuttle

becausethatiswheretheproductswillbe
consumed;acetylcoA;TCAcycleofketone
bodyproduction

fattyacid(acylcoA)oxidationtoacetylcoA;
increaseindicarboxylicacids,anddecreased
glucoseandketones

lipoproteinlipasestakeFAfromVLDLandchylomicrons
whicharethencombinedwithglycerol3P(acarb
intermediate)tomaketriacylglycerol

synthesisinliverandthenhydrolysisofchylomicronsand
VLDLsbycapillarylipoproteinlipaseinperipheraltissue;
adiposetissuebylipases

falseonlymuscleandbrainhavetransferaseenzymethatconverts
ketonebodiestoacetylcoA

whereareketonebodiesmade?whatistherate
limitingenzyme?whataretheymadefrom?

whatdoesthebraindowithketone
bodies?

metabolizesthemwithtransferaseenzymestoacetylcoA

whatarethreeketonebodies?whichisnotdetected
inurine?whichdoyouseemostinalcoholic
ketoacidosis?

mitochondriainliver;HmGCoAsynthetase;
aminoacidsandbetaoxidationofFA

bhydroxybutyrate,acetone,acetoacetate;
betahydroxybutyrate;betahydroxybutyrate

oxaloacetatedepletedforgluconeogenesis;excessNADH

whatcausesfruityodorinketoacidosis?

howmanycaloriesdoesonegramofproteingenerate?carb?fat?

T/FHMGcoAreductaseisusedin
KBsynthesis

StoredATP,creatinephosphate,anaerobic
glycolysis;asdistancesincrease,ATPisobtained
fromadditionalresources;aboveplusoxphos

glycogenandFFAoxidationglucoseconservedforfinalsprinting!

whataretheprioritiesinfastingand
starvation?

tosupplysufficientglucosetothebrainandtheRBCsand
topreserveprotein

whatisthesourceofATPafterameal?

whatisthesourceofATPwhenyou
arefasting(inbetweenmeals)?

glycolysisandaerobicrespiration

hepaticglycogenolysis(mostly),alsohepatic
gluconeogenesis,adiposereleaseofFFA(minor)

afterhowlongofstarvationdoglycogen
reservesdeplete?afterthisoccurs,howare
bloodglucoselevelsmaintained?

1day;adiposereleaseofFFA,hepatic
gluconeogenesisfromperipheraltissuelactateand
alanineandfromadiposetissueglyceroland
propionylcoA

whatdoesadiposetissuecontributeforhepatic
gluconeogenesisduringstarvation?muscle?

afterday3ofstarvation,whatisthe
mainsourceofenergy?

4,4,9

falseHMGcoAsynthase;cholesterolsynthesisusesHMG
coAreductase

ina100metersprintwhatisusedas
energy?ingeneralwhatistherulewith
exercise?whatisusedin1000meterrun?

whatisusedina
marathon?

acetone

whatistheratelimitingstepincholesterol
synthesis?whatdoesitdo?

glycerolandpropionylcoA;lactate
andalanine

adiposetissueforbrainandheart(ketonebodies)andthengo
tovitalproteindegradation(organfailure)

HMGCoAreductase;convertsHMGCoAto

mevalonate

howmuchofplasmacholesterolisesterified?and
bywhat?

whatdostatinsinhibit?

2/3;LCAT(lecithincholesterol
acyltransferase)

HMGCoAreductase