00HR0CK At0 SClAttA

l000 6R0uPlt6 SYSTFH

Croup # 3
Croup Members
W Marlna 8rldgemohan
W nemlsscla !ordan
W uevanand 8amnarlne
W 1lffany klng
uLllne
W uombrock blood grouplng sysLem
PlsLory
roperLles
opulaLlon ulsLrlbuLlon
henoLype ulsLrlbuLlon
Cllnlcal Slgnlflcance
8lochemlsLry
uLllne
W Sclanna blood grouplng sysLem
ClasslflcaLlon
PlsLory
roperLles
Cllnlcal Slgnlflcance
opulaLlon ulsLrlbuLlon
henoLype ulsLrlbuLlon
8lochemlsLry
uombrock 8lood Crouplng SysLem
W 1he uombrock blood group sysLem ls Lhe classlflcaLlon
of human blood based on Lhe presence of cerLaln
glycoproLelns called glycosylphosphaLldyllnoslLol (Cl)
anchored proLeln
W 8epresenLed by Do
W (uo) anLlgen was flrsL dlscovered on Lhe surface of 88C
uombrock AnLlgens
1he assoclaLed anLlgens of Lhls blood group
sysLem are
W uo
a
W uo
b
W Cy
a
(Cregory anLlgen)
W Py (Polley anLlgen)
W !o
a
(!oseph anLlgen)
PlsLory
W AnLlbodles Lo Lhe uombrock anLlgen uo
a
were
dlscovered ln 1963 ln a paLlenL who had
recelved a blood Lransfuslon
W ln 1973 a second uombrock anLlgen (uo
b
) was
ldenLlfled
PlsLory ConL'd
1he uombrock blood group was expanded ln Lhe
1990s
lncluded Lhree oLher anLlgens namely
W Cy
a
(Cregory anLlgen)
W Py (Polley anLlgen)
W !o
a
(!oseph anLlgen)
n8 All occurred on Lhe same proLeln as Lhe uo
anLlgens
roperLles of Lhe uombrock AnLlgen
W lL ls encoded by Lhe gene @(Au
rlbosylLransferase 4)
W ocaLed on Lhe shorL arm of chromosome
12p132p121
roperLles ConL'd
W 1he Cl ls embedded ln Lhe membrane of
uombrockexpresslng cells hereby enabllng Lhe
proLeln and lLs anLlgenconLalnlng resldue Lo
remaln exposed on Lhe ouLslde surface of Lhe
cells
W number of amlno aclds ls 314
W CeneLlc varlaLlons ln @LhaL resulL ln an
alLered amlno acld sequence of Lhe encoded
proLeln glve rlse Lo Lhe dlfferenL uombrock
Carrler roLeln
opulaLlon ulsLrlbuLlon
W SLudles lndlcaLed LhaL
W 63 of norLhern Luropeans carry Lhe uo
a
anLlgen
W uo
b
anLlgen has an lncreased lncldence ln
Afrlcans and Aslans
W Cy
a
Py and !o
a
anLlgens have been esLlmaLed
Lo occur ln more Lhan 99 of lndlvlduals
henoLype ulsLrlbuLlon
9otyp Do
a
Do
b
Gy
a
ny Io
a
Caucas|as 8|acks
Do (a+b) + 0 + + + 18 11
Do (a+b+) + + + + + 49 44
Do (ab+) 0 + + + + 33 43
Gy (a) 0 0 0 0 0 8are 8are
ny 0 wk wk 0 0/wk 0 8are
Io (a) wk 0/wk + wk 0 0 8are
uombrock AC ulsLrlbuLlon ln 8ody
AparL from Lhe surface of Lhe 88C uo
a
ls found
on
W ymphocyLes and lymph nodes
W 8one marrow
W 1lssues of Lhe spleen ovarles LesLes
lnLesLlnes and feLal hearL
n8 1he expresslon of uombrock anLlgens ls
null henoLype
W 1he absence of uombrock anLlgens occur
because of loss of Clanchored proLelns from
Lhe surface of 88C resulLlng ln uombrocknull
phenoLype
W oss of Lhe proLeln resulLs ln aroxysmal
nocLurnal Pemogloblnurla (nP) (88C
undergo premaLure desLrucLlon by lmmune
cells )
AnLlbodles and Cllnlcal Slgnlflcance
W uombrock anLlbodles ls predomlnanLly lgC
W uo noL acLlvaLe complemenL sysLem
W 1hey have noL been assoclaLed wlLh cllnlcal
PemolyLlc ulsease of newborn (Pun)
W Powever anLlbodles have been assoclaLed
wlLh severe PemolyLlc 1ransfuslon 8eacLlon
(P18)
8lochemlsLry
W 8eslsLanL Lo papaln or flcln and chloroqulne
W SenslLlve Lo Lrypsln chymoLrypsln pronase
or u111200
Summary
81 Nam
(81
Numb)
Comosom
Locat|o
G Nam
GN (81)
9|ct
1opo|ogy
(Numb of
am|o ac|s)
Compot
Nam (CD
umb)
Assoc|at
8|oo Goup
At|gs
Dombock
(014)
12p132p121 u (u)
Clllnked
(314)
uo
glycoproLeln
A81 4
uo
a
uo
b
Cy
a

Py !o
a
SClAttA SClAttA l000 6R0uPlt6 l000 6R0uPlt6
SYSTFH SYSTFH
Sclanna 8lood Croup SysLem
A polymorphlc blood group anLlgen LhaL are
deLermlned by eplLopes on Lhe surface of red blood
cells
Sclanna 8lood Croup conslsLs of anLlgens
W Sc1
W Sc2
W Sc3
W 8d ( 8adln)
Sclanna 8lood Croup SysLem
LsLabllshed 1974
W lS81 SC
W lS81 # 013
W Lher name Sc
Lxpresslon
W Lher blood cells Weakly expressed ln
leukocyLes Lhymus lymph nodes spleen
W 1lssue leLal llver bone marrow ln adulLs
ClasslflcaLlon
1hese anLlgens are furLher classlfled as by Lhelr
frequencles
W Sc2 and 8d are consldered as low frequency
W Sc1 and Sc3 are boLh hlgh frequency
PlsLory
W ln 1962 an anLlbody Lo a hlghprevalence
anLlgen anLlSm was descrlbed by SchmldL
W 1963 low lncldence anLlbody (anLlSc2/8u
a
)
was dlscovered by Anderson
PlsLory
W 8d (8adln) was recenLly added Lo Lhe Sclanna
sysLem Powever lL was descrlbed by Lhe
expresslon of Sclanna sysLem anLlgens on
L8MA
W 8d ls Lhe resulL of a muLaLlon ln kMAl LhaL
produces a change ln Lhe expressed proLeln
(ro60Ala)
W lmmunobloLLlng wlLh anLl8d had shown LhaL
roperLles of Lhe anLlgen
W 1he anLlgens are carrled on human eryLhrold
membraneassoclaLed proLeln (L8MA)
W ConslsL of 473 amlno aclds
Carrler roLeln
AssoclaLed gene
W Sclanna glycoproLeln ls Lhe anLlgen sLrucLure
LhaL encodes Lhe SC gene
W ocaLed on chromosome 1 p34
AnLlbody and Cllnlcal Slgnlflcance
W lgC ls Lhe respecLed anLlbody for Sclanna 8lood group
W AnLlbodles are produced because of 88C sLlmulaLlon
Lhrough Lransfuslon or pregnancy
W Can acLlvaLe complemenL sysLem
W Pave been reporLed Lo cause mlld (Pun)
Cllnlcal Slgnlflcance
W lL ls noL known Lo cause cllnlcal Lransfuslon problems
W AnLlSc1 ls a rare lgC anLlbody reacLlve by Lhe anLl
globulln LesL
Slbllngs of paLlenLs maklng Lhe anLlbody should be LesLed
for compaLlblllLy and paLlenLs encouraged Lo donaLe blood
for long Lerm sLorage when Lhelr cllnlcal condlLlon permlLs
Cllnlcal Slgnlflcance
W Slnce Lhe Sc2 anLlgen has a lowlncldence Lhe greaL
ma[orlLy of donor blood samples wlll be compaLlble
W AnLlSc2 ls usually lgC and reacLlve by Lhe anLl globulln
LesL AbouL 1 of random donor red cell samples are
Sc2+
opulaLlon ulsLrlbuLlon
W n a hlgh frequency anLlgen found ln greaLer
Lhan 99 of mosL populaLlons
W 1he frequency of Sc2 ls abouL 1 of norLhern
Luropeans buL Lhe frequency ls much lower ln
oLher populaLlons
W Sc12 ls more common ln MennonlLe
W n a hlgh frequency anLlgen found on all
cells excepL ln persons who Lype Sc12
henoLype ulsLrlbuLlon
henoLypes Caucaslans 8lacks
Sc l2 99 100
Sc l 2 1 0
Sc l 2 very rare 0
Sc l 2 8d+ very rare very rare
Sc l 2 8d+ very rare 0
8lochemlsLry
W 1he Sc1 and Sc2 anLlgens can be LreaLed wlLh
u11
W 8eslsLanL Lo flcln/papaln Lrypslnr
chymoLrypsln and Chloroqulne
W 1he 8d anLlgen dlffers ln LhaL Lhe effecL of
Lrypsln and chymoLrypsln LreaLmenL ls
varlable
Summary
81 Nam
(81
Numb)
Comosom
Locat|o
G Nam
GN (81)
9|ct
1opo|ogy
(Numb of
am|o ac|s)
Compot
Nam (CD
umb)
Assoc|at
8|oo Goup
At|gs
c|aa
(013)
1p34 L8MA (SC) 1ype l (473) L8MA
Sc1 Sc2 Sc3
8d
8eferences
W kara k8 2010 1he Puman 8ody lbysly oo cltnotlo 1
sL
ed
8rlLannlca LducaLlonal ubllsherpp110111
8eLrleved here
hLLp//booksgooglecom/books?ldlbZwlu1mMCpgA110dqdombrock+
blood+grouplng+sysLemhlenel3_qv1u2oPZPqgCe![dPCACsaxolbook_
resulLcLresulLresnum2ved0CukC6ALwAC#vonepageqffalse
W !ohn Ceer eL al 2009 WlnLrobe's Cllnlcal PaemaLology 12
Lh
ed
lpplncoLLpp 672
8eLrleved here
hLLp//booksgooglecom/books?ld68enzuu78vgCpgA644dqdombrock+bl
ood+grouplng+sysLemhlenel3_qv1u2oPZPqgCe![dPCACsaxolbook_re
sulLcLresulLresnum3ved0Cu0C6ALwAg#vonepageqdombrock20blo
od20grouplng20sysLemffalse
8eferences
W uelores Mallory uM eL al 2003 lmmunohemaLology too
b ts sety oo enotlo vol 21 Lhe Amerlcan 8ed
Cross pp 70
8eLrleved here
hLLp//a1881gakamalneL/7/1881/26640/v0001/redcrossdownloada
kamalcom/26640/pubs/lmmuno/21_2_03pdf
W hLLp//scarfex[oveprohosLlngcom/blood/13hLml
W
hLLp//blood[ournalhemaLologyllbraryorg/conLenL/96/7/262
1full