Amino Acid Metabolism

rokiheyu's version from 2015-12-23 11:30

Section 1

Question Answer
transaminaseAA + alpha-ketoacid <==> alpha-ketoacid + AA
glutamate dehydrogenaseglutamate ==> alpha-ketoglutarate + NH4+
Type I primary oxaluriadeficient transaminase ==> increased oxalate ==> renal failure
histidinemiadeficient histidase ==> possible mental retardation
methylmalonyl CoA mutase deficiencyincreased propionyl CoA, OAA ==> forms a toxin ==> failure to thrive, dehydration, delayed development, seizures
homocysteinuriadeficient cystathione beta-synthase ==> increased homocysteine and methionine ==> lens dislocation, mental retardation, skeletal abnormalities, neurologic abnormalities
maple syrup urine diseasedeficient branched chain AA dehydrogenases ==> accumulation of Val, Ile, Leu ==> urine smells like maple syrup, mental retardation, poor nerve myelination
phenylketonuriadeficient phenylalanine hydroxylase ==> phenylalanine accumulation ==> musty odor urine, mental retardation
alkoptanuriadeficient homogentisate oxidase ==> homogentisic acid accumulation ==> dark colored pigments in urine, fibrous tissue, eventual arthritis
Isovaleric acidemiadeficient isovaleryl CoA dehydrogenase ==> deficient leucine degradation ==> neuromuscular irritability, mental retardation, sweaty feet

Section 2

Question Answer
Serine synthesisfrom 3-phosphoglycerate
Glycine synthesisfrom serine
Cysteine synthesisfrom serine, methionine
Alanine synthesisfrom pyruvate
Aspartate synthesisfrom OAA
Asparagine synthesisfrom Aspartate
Glutaratefrom alpha-ketoglutarate
Glutaminefrom glutamate
Prolinefrom glutamate
Argininefrom glutamate
Tyrosinefrom Phenylalanine

Section 3

Question Answer
Histidine metabolismalpha-ketoglutarate (glucogenic)
Arginine metabolismalpha-ketoglutarate (glucogenic)
Proline metabolismalpha-ketoglutarate (glucogenic)
Glutamine metabolismalpha-ketoglutarate (glucogenic)
Glutamate metabolismalpha-ketoglutarate (glucogenic)
Alanine metabolismpyruvate (glucogenic)
Cysteine metabolismpyruvate (glucogenic)
Glycine metabolismpyruvate (glucogenic)
Serine metabolismpyruvate (glucogenic)
Threonine metabolismsuccinyl CoA via propionyl CoA (glucogenic); Acetyl CoA (ketogenic)
Isoleucine metabolismsuccinyl CoA via propionyl CoA (glucogenic); Acetyl CoA (ketogenic)
Methionine metabolismsuccinyl CoA via propionyl CoA (glucogenic)
Valine metabolismsuccinyl CoA via propionyl CoA (glucogenic)
Tyrosine metabolismfumarate (glucogenic); Acetoacetate (ketogenic)
Phenylalanine metabolismfumarate (glucogenic); Acetoacetate (ketogenic)
Aspartate metabolismOAA (glucogenic)
Asparagine metabolismOAA (glucogenic)
Lysine metabolismAcetyl CoA (ketogenic)
Tryptophan metabolismAcetyl CoA (ketogenic)
Leucine metabolismAcetyl CoA, Acetoacetate (ketogenic)

Section 4

Question Answer
Creatine derived fromGlycine + Arginine + SAM
Glutathione derived fromGlutamine + Cysteine + Glycine
Nitric Oxide derived fromL-arginine ==> citrulline byproduct
GABAGlutamate decarboxylation
HistamineHistidine decarboxylation
Ceramide/ SphingolipidsSerine + palmitoyl CoA ==> decarboxylation
SerotoninTryptophan decarboxylation
DopamineTyrosine decarboxylation
NE, EpinephrineTyrosine