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21 Cards in this Set
- Front
- Back
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carbamoyl phosphate synthetase I
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CO2+2ATP+NH4 -->carbamoyl phosphate
*requires N acetyl glutamate in mitochondrial matrix |
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carbamoyl phosphate synthetase II
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in cytosol
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L orthinine transcarbamyolase
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orthinine + carbamoyl phosphate => citrulline
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arginosuccinate synthase
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citrulline + aspartate + ATP => argininosuccinate + AMP
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argininosuccinase
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cleavage of argininosuccinate to arginine and fumarate (fumurate is converted back to malate)
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arginase
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arginine --> orthinine + urea
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N-acetylglutamate synthase
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acetyl COA + glutamate --> N acetylglutamate + COA
activated by Arginine |
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ketogenic amino acids
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Leucine and lysine
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Glucogenic and Ketogenic
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Tyrosine
Isoleucine Phe Trp (PITT) |
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Glucogenic AA
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yield pyruvate or one of the other intermediates of the TCA cycle
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Ketogenic AA
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yields acetoacetate, acetyl COA or acetoactyl COA
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homocystinuria
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caused by cystathione synthase or Vit B6 deficiency
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cystathioninuria
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caused by gamma synthionase or vit B6 deficiency
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methylmalonic acidemia
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caused by methylmalonyl COA mutase
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branched chain alpha AA aminotransferase
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Leu, Ile and Val are transaminated to alpha keto acids
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Maple Syrup Urine disease
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mutations in enzymes that catalyze the oxidative phosphorylation of branched alpha keto acids (in urine)
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Alkaptonuria
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involves deficiency in homogentisate oxidase
homogentisate in urine leads to dark color when oxidized |
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tyrosinase
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key enzyme in melanin formation
deficiency leads to albinism |
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AA that participate in creatinine synthesis
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Arg, Gly and Met
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AA that participate in creatinine synthesis
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Arg, Gly and Met
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Tyrosine Hydroxylase
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tyrosine -->DOPA
*requires BH4 |
