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Ketogenic AA

USCSOM: Catabolism of Ketogenic Amino Acids

Which AA is the only strictly ketogenic-branched chain amino acid? Leu
Branched chain amino acids undergo carboxylation to form CoA derivatives via what enzyme? alpha-keto acid dehydrogenase
What 7 amino acids from Acetyl CoA? Leu, Ile, Lys, Tyr, Thr, Phe, Trp
What enzyme converts phenylalanine into tyrosine? phenylalanine hydroxylase
What is a deficiency in the phenylalanine hydroxylase called? PKU- phenylketouria
What is the fate of the catabolism of Trp? Kynurenine, Formate, Alanine, Niacin
Saccharopine is an intermediate in catabolism of what AA? Lys
Genetic abnormalities in saccharopine can lead to what two causes in the blood? hyerlysinemia, hyperammonemia
What is the chance of PKU in live births? 1:10,000
Elevated Phe can also lead to what other metabolites being elevated? phenylpyruvate, phenylacetate, phenyllactate
What are some symptoms of PKU babies? delayed psychomotor maturation, tremors, seizures, eczema, hyperactivity
Patients with PKU that develop neurological symptoms despite diet restrictions have a deficiency in what enzyme? BH2 reductase (DHPR)
What 2 things causes malignant hyperphenylalaninemia? DHPR deficiency or GTP biosynthesis deficiency
What is homocystinuria? elevated homocysteine and methionine
What is the cause of homocystinuria? defect in cystathionine synthase or cystathionase
What is the treatment for homocystinuria? reduce Met intake and vit B6
What is the cause of Cystinuria? defect in the intestinal epithelial transport cells for Cys2, Lys, Arg, Orn
What is the TX for Cystinuria? increase fluid volume and pH in the urine
What is cystinosis? a rare disorder caused by defective Cys2 carrier across lysosomal membranes
What is the cause of Maple Syrup Urine Disease? defect in dehydrogenase that decarboxylates branched-chain AAs (Leu, Ile, Val)
What are clinical signs of alcaptonuria? darkening of urine on standing in air
What causes alcaptonuria? lack of homogentisate oxidase involved in the catabolism of Tyr
What is significant about tyrosinemias? nothing, they are benign
What are the 4 known defects in Tyrosine catabolism? Type I tyrosinemia
What is Kwashiorkor? lack of protein in the diet
What are symptoms of kwashiorkor? muscle wasting, decreased plasma proteins, edema
What causes hartnup disease? impaired transport of neutral AAs across intestinal and renal epithelial cells
What are symptoms of hartnup disease? like pellegra (4D's)
What 2 AAs are thought to be strictly ketogenic including both branched and non-branched AAs? Leu and Lys
What is BH4? tetrahydrobiopterin
What is BH2? dihydrobiopterin
What AA competitively inhibits liver arginase? What does it cause? Lys; hyperammonemia
What AA becomes an essential dietary AA with PKU? Tyr
Created by: jlellerm
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