MODULE 6 : AMINO ACID CATABOLISM Flashcards
List of glucogenic amino acids
PYRUVATE KETOGLUTARATE SUCCINYL COA FUMARATE OXALOACETATE
First step in amino acid catabolism
REMOVAL OF AMINO NITROGEN
Except for PROLINE, HYDROXYPROLINE, LYSINE & THREONINE
List of ketogenic amino acids
ACETYL CoA
ACETOACETATE
Amino acids when catabolized leads to pyruvate
CYS SER ALA GLY THR TRP
Amino acids when catabolized leads to oxaloacetate
ASP
ASN
Amino acids when catabolized leads to ketoglutarate
ARG GLU GLN HIS PRO
Amino acids when catabolized leads to fumarate
PHE
ASP
TYR
Amino acids when catabolized leads to Succinyl CoA
VAL
ISO
MET
Amino acids when catabolized leads to acety CoA / acetoacetate
ISO LEU THR LYS PHE TYR TRP
Failure to catabolize oxalate leads to?
HYPEROXALURIA
Alanine is catabolized to pyruvate by what process
TRANSAMINATION
Asparagine is hydrolyzed to what amino acid before it is further catabolized to oxaloacetate
ASPARTIC ACID
Amino acids used to determine folic acid deficiency
HISTIDINE + FIGLU (N-formiminoglutamate)
Enumerate first three steps on the catabolism of branched chain amino acids
- TRANSAMINATION
- OXIDATIVE DECARBOXYLATION
- DEHYDRATION
Active form of methionine
SAM
S Adenosyl L Methionine
Methionine is converted to ___________ which is then further converted to Succinyl Coa
A. A - ketobutyrate
B. Propionyl Coa
C. Methylmalonyl Coa
A. A - ketobutyrate
Isoleucine is converted to ___________ which is then further converted to Succinyl Coa
A. A - ketobutyrate
B. Propionyl Coa
C. Methylmalonyl Coa
B. Propionyl Coa
Valine is converted to ___________ which is then further converted to Succinyl Coa
A. A - ketobutyrate
B. Propionyl Coa
C. Methylmalonyl Coa
C. Methylmalonyl Coa
____________ is converted to a - keyobutyrate which is then further converted to Succinyl Coa
A. Methionine
B. Isoleucine
C. Valine
A. Methionine
____________ is converted to propionyl coa which is then further converted to Succinyl Coa
A. Methionine
B. Isoleucine
C. Valine
B. Isoleucine
____________ is converted to methylmalonyl coa which is then further converted to Succinyl Coa
A. Methionine
B. Isoleucine
C. Valine
C. Valine
What AA is degraded via the formation of Saccharopine?
Lysine
Carbon atoms of side chain and aromatic ring of TRYPTOPHAN is completely degraded via what pathway?
Kynurenine-anthranilate pathway
Kynurenine-anthranilate pathway is for degradation of what AA?
A. K
B. Y
C. W
D. S
C. W
An iron porphyrin metalloprotein involved in the initial reaction in the degradation of tryptophan
Tryptophan Oxygenase
Tryptophan oxygenase is inducible in the liver by?
Adrenal corticosteroid and Tryptophan
The action of Tryptophan Oxygenase is inhibited by?
Nicotinic Acid Derivatives (NADPH)
This AA is purely Ketogenic
Leucine
What are the 3 branched Chain AA?
A. KYL
B. VIL
C. QRE
D. FES
B. V I L
Aminoacidopathy:
Tyrosine Catabolism is impaired
Alkaptonuria
Aminoacidopathy:
Alkaptonuria: what is the deficient enzyme?
Homogentisate oxidase
Aminoacidopathy:
Presence of homogentisate in the urine is a character of what condition?
Alkaptonuria
Aminoacidopathy:
Impaired conversion of F to Y
Phenylketonuria
Classic PKU condition has a defect on what enzyme?
A. Tetrahydrobiopterin Synthase
B. Dihydrobiopterin Synthase
C. Phenylalanine Hydroxylase
C. Phenylalanine Hydroxylase
Aminoacidopathy:
Results from impaired intestinal and renal transport of neutral AA including W
Hartnup Disease
Aminoacidopathy:
Results from the absence of branched chain A - ketoacid dehydrogenase complex
MSUD
