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