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20 Cards in this Set
- Front
- Back
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Pepsin
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N-terminus of Ile, Phe, Val
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Trypsin
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At Lys, Arg
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Elastase
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At Ala, Gly
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Chymotrypsin
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At Phe, Trp, Tyr
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Phenyalanine hydroxylase
*Co-Factor? |
Phe -> Tyr
*Tetrahydrobiopterin |
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Phenylalanine aminotransferase
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Phe -> Phenylpyruvate
Phenylpyruvate -> Phenylacetate, phenyllactate *Only in PKU |
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Tyrosine aminotransferase
*Co-factor? |
Tyr -> Hydroxyphenylpyruvate
*Concurrent Glu -> Alpha-KG *Requires PLP |
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Tyrosinemia II
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Tyrosine aminotransferase deficiency
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Hydroxyphenylpyruvate diooxygenase
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Hydroxyphenylpyruvate -> Homogenistate
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Tyrosinemia III
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Hydroxyphenylpyruvate diooxygenase deficiency
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Homogenistate-1,2,-diooxygenase
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Homogenistate -> Maleyloacetoacetate
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Alkaptonuria
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Homogenistate-1,2-diooxygenase deficiency
*Black urine, changes color when exposed to oxygen *Causes calcification, arthritis in spine, joint capsules |
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Maleyloacetoacetate isomerase
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Maleyloacetoacetate -> Fumarylacetoacetate
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Fumarylacetoacetase
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Fumarylacetoacetase -> Fumarate, acetoacetate
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Tyrosinemia I
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Deficiency in fumarylacetoacetase
*Requires liver transplant *NTBC |
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3-ketoacyl CoA transferase
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Acetoacetate -> Acetoacetyl CoA
*Succinyl CoA serves as CoA donor |
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Branched chain aminotransferase
*Co-factor? |
Val, Leu, Ile -> alpha-ketoacids
*Requires PLP |
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Alpha-ketoacid dehydrogenase complex
*Co-factor(s) |
Alpha ketoacids -> Acyl CoA derivatives
*Thiamine, CO2, ATP |
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Maple syrup urine disease
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Alpha-ketoacid dehydogenase deficiency
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Branched chain amino acid final products
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Val -> Succinyl CoA
Ile -> Succinyl CoA, acetyl CoA Leu -> Acetyl CoA, Acetoacetyl CoA |
