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Ketogenic AA
USCSOM: Catabolism of Ketogenic Amino Acids
| Question | Answer |
|---|---|
| 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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