metabolism_lecture_10+11

metabolism_lecture_10+11 - Fate of the carbon skeleton for...

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Fate of the carbon skeleton for individual amino acids Seven to  acetyl-CoA Leu, Ile, Thr, Lys, Phe, Tyr, Trp Six to  pyruvate Ala, Cys, Gly, Ser, Thr, Trp Five to  α -ketoglutarate Arg, Glu, Gln, His, Pro Four to  succinyl-CoA Ile, Met, Thr, Val Two to  fumarate Phe, Tyr Two to  oxaloacetate  Asp, Asn All 6 products enter the CAC gluconeogenesis, ketogenesis, or  oxidation to CO 2 +H 2 O Only Leucine and Lysine are  exclusively ketogenic
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N, D        OA + Citrulline arginine +  fumarate urea cycle:
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W, A, T, G, S, C        pyruvate T = glucogenic +       ketogenic
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W, K, F, Y, L, I       Acetyl-CoA W, F, Y, I = glucogenic +       ketogenic thiolase
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R, P, H, E, Q             α -KG
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M, T, I, V           succinyl-CoA
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Tryptophan as an important precursor  (5-hydroxytryptamine)
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cp. toxicity  of NH 4 +   Human genetic disorders
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Inheritable human diseases linked to catabolic pathways for Tyr and Phe phenylketonuria
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Phenylketonuria When phenylalanine hydroxylase is deficient, Phenylalanine gets deaminated. Phenylpyruvate accumulates in blood, tissue, and urine. Phenyllactate and Phenylacetate found in urine. Phenylketonuria leads to: Mental retardation Smaller brain, Reduced myelination of nerves Hyperactive reflexes Also: high [Phe] inhibits melanine synthesis          -> lighter skin) Treatment: -> phenylalanine reduced diet
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Alkaptonurina Deficiency in homogentisate 1,2-dioxygenase Accumulation of homogentisate in urine Its oxidation and polymerization leads to dark color “Inborn errors of metabolism” Zacutus Lusitanus, 1649
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Chapter 18: Summary Amino acids from protein are an important  energy source  in carnivorous animals Catabolism of amino acids involves transfer of the amino group via PLP-dependent  aminotransferases to a donor such as  α -ketoglutarate  to yield L-glutamate L-glutamate and L-glutamine  can be used to synthesize new amino acids, or it can dispose of  excess nitrogen as ammonia  In most mammals, toxic  ammonia  is quickly recaptured into carbamoyl phosphate and passed  into the  urea cycle
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Chapter 19: Oxidative phosphorylation Electron transport chain in mitochondria Building up the the proton-motive force Synthesis of ATP in mitochondria Key topics: 
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Energy from reduced fuels is used to synthesize ATP Carbohydrates, lipids, and amino acids are the main  reduced fuels  for the cell Electrons from reduced fuels are used to reduce NAD +  to  NADH  or FAD to  FADH 2 .
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