Biochemistry Second Year

1. Diseases
Biological
Importance
Synthesized fromCatabolized intoAmino Acid
well fed
Glucose pyruvate alanine
Hunger
Alanine pyruvate glucose
(Glucose – alanine cycle)
Pyruvate
Glutamate-pyruvate
aminotransferase
(alanine transferase ALT)
Pyruvate
(Alanine
Transferase)
Alanine
Non-essential
Glucogenic
1) 3-phosphoglycerate
2) Glycine
(hydroxymethyltransferase)
1)Pyruvate
(serine dehydratase
)
2)
3-phosphoglycerate
Serine
non-essential
glucogenic
1)Pyruvate
(threonine
dehydrogenase )
2) succinyl CoA
Ser)thrdehydratase
3) AcetylCoA
(ketogenic)
Therionine
Essential
Both
1)
Nonketotichyperglycinemia
2) oxalate kidney stones
1) serine
Hydroxymethyltransferase
2) threonine
1) glyoxalate
(transaminase)
2) CO2 & NH3
Glycine cleavage
complex
Glycine
Non-essential
glucogenic

2. 1)homocysteinuria
2) high blood level
of homocysteine
3) cystathionineuria
methionine+ serine
A- Pyruvate
1) desulfurase
2) dioxygenase
3) By 2 cysteine
molecules
B-
marcaptopyruvate
Cysteine
Non-essential
Glucogenic
1)Glutamate-oxaloacetat
aminotransferase
Asparatatetransferase AST
2) Asparagine
Asparaginase
1) Oxaloacetate
(aspartate
aminotransferase)
2) fumarate ( in urea
cycle )
Asparatate
Non-essential
Glucogenic
Aspraginase treatment.
(asparaginessynthetase)
Amidation of aspartate
Asparaginessynthetase
Aspartate
Oxaloacetate
Asparaginase
Asparagine
non-essential
glucogenic
GABA
Decarboxylase
1) NH4+a-ketoglutarate
(Glutamate dehydrogenase)
2) glutamine
( glutaminase )
1)a-ketoglutarate
(Glutamate
aminotransferase)
(Glutamate
dehydrogenase)
Glutamate
Non-essential
glucogenic

3. Glutamate
(Glutamine synthetase)
Glutamate
(αketoglutarate)
Glutamine
Non-essential
glucogenic
Phosphoceratine cearatine Ceratinine
(urine)
α-ketoglutarate
α-ketoglutarate
(Arginase)
Arginine
Non-essential
glucogenic
Succinyl CoA
Valine
Essential
glucogenic
1)Acetyl CoA
2) propionyl CoA -
>succinyl CoA
Isoleucine
Essential
both

4. a-ketoglutarateα- ketoglutarate
Proline
Non-essential
glucogenic
Hitidinemia
Histamine formation
(Histidine decarboxylase)
Glutamate
(α- ketoglutarate)
Histadinase
Histidine
Essential
glucogenic
Succinyl CoA
Methionine
Essential
glucogenic
Phenylketouria (PKU)
1)fumarate
2) acetoacetate
3) tyrosine
3-phenylalanine
hydroxylase
Phenylalanine
Essential
Both

5. Tyrosinemia
Alkaptonuria
Albinism
1)DOPA
2)Dopamine
(related to
parkinson's
disease in brain)
3)norepinephrine
& epinephrine
4/Melanine
Phenylalanine
(Phenylalanine Hydroxylase)
1) fumarate
2)acetoactate
Tyrosine
Non-essential
both
1) brain
neurotransmitter
serotinine
2) melatonine
3) kynurenine
1)glutarate
2) acetoacetyl CoA
Tryptophan
Essential
both
1) acetoactate
2) acetyl CoA
Leucine
essential
ketogenic
1) hyperlysinemia
2)familial lysinuric
protein intolerance
1) acetoactate
2) acetyl CoA
Lysine
Essential
ketogenic

6. Valine , isoleucine &leucine are branched-chain amino acid ( BCAAs).
Diseases of metabolism of BCAAs
Deficiency in ketoacid dehydrogenases lead to maple syrup urine
Enzyme deficiency of lateral reactions gives sweaty feet urine smell &cat urine smell
each orange color mean name of enzyme
both = ketogenic&glycogenic
NOTE: NAMES OF ENZYMES IN TABLE ARE NOT REQUIRED………