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INTRODUCTION
Under normal circumstances, they are not used for energy production
Screening tests
Treatment
Urea synthesis
All tissues have some capability for synthesis of the non-essential amino acids
Transamination
Pyruvate +
Transamination
Most important
Synthesis
Broad Substrates
(Cannot act with
of nonionizable side
essential
amino acids
Exception:
Schiff base, imine
Lysine,
intermediate hydrolyzes
to form keto acid and
Threonine,
ammonia
Proline
Glucogenic
Ketogenic
Leucine, Lysine
Glucogenic and Ketogenic
All twenty common amino acids are converted to only seven compounds
1. Pyruvate
2. -Ketoglutarate
3. SuccinylCoa
4. Fumarate
5. Oxaloacetate
6. Acetyl CoA
7. Acetoacetyl CoA
Glycine and All Three-Carbon Amino Acids are Converted to Pyruvate
Deficiency of
Proline
GPT, PLP
[O2]
Cysteine
Pyruvate + SO3-2
Transaminase/Desulfin
Cyssulfinate
ase
Transamina
se
3-Mercaptopyruvate
S
transferas
Glycine Cleavage
Threonine e
Acetaldehyde
Dehydrogen
ase
Glycine
H4F
SerHydroxymeth
yl
THF
Acetyl
Thiokinas
CoAe
Acetate
Serine
Deficiency of
HistidinaseHistidinemia
UrocanaseUrocanic Aciduria
Histidine Loading Test Folic Acid Level
Catabolism of Methionine
Arthritis
Darkening of urine
on standing
Tryptophan Pyrrolase
or Tryptophan
Oxygenase is a
hemoprotein
Kynureninase
requires pyridoxal
Mental retardation
Ketoacidosis
phosphate
Tryptophan
load
diagnostic
of Vitamin
B6
deficiency
Hartnup
disease reflects impaired intestinal and renal
transport of Trp
Phenylketonuria (PKU)
Deficiency of
Phenylalanine
Hydroxylase
Autosomal
recessive
Neurological
signs and
symptoms
Mousy urine
(Phenylacetate)
The end
product
phenylpyruvate
accumulates in
liver, blood, and
urine
Phenylacetate and phenyllactate are also found in urine
Alkaptonuria
Autosomal recessive
Catabolism of
Lysine
Aspartate
Glutamin
e
Purine
Pyrimidin
Polyamines
Stabilized
Arginin
e
Nitric Oxide (NO)