Term
What enzyme is defective in juvenile Parkinson's? |
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Definition
Parkin- an E3-ubiquitin-ligase |
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Term
What does the HPV virus do? |
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Definition
encodes an E3-ubiquitin ligase that targets p53 for degradation |
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Term
What is defective in Huntington's and what does it due |
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Definition
defective htt protein binds to proteasomes and inhibits degradation of other proteins which accumulate and cause neurodegeneration |
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Term
What do parietal cells of stomach release |
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Definition
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Term
what do chief cells of stomach release |
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Definition
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Term
Where does trypsin cleave |
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Definition
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Term
Where does elastase cleave cleave |
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Definition
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Term
Where does chymotrypsin cleave |
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Definition
after cyclic, aromatic AA's |
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Term
Three AA's used for 10% of nrg for extrahepatic tissues |
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Definition
valine, leucine, and isoleucine |
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Term
Phenylalanine hydroxylase |
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Definition
converts phenylalanine to tyrosine |
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Term
What enzyme do phenylketonuria (PKU) patients use to metabolize phenylalanine |
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Definition
aminotransferase; converts pyruvate to alanine in process |
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Term
tyrosine aminotransferase- deficiency |
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Definition
1. tyrosine--> p-hydroxyphenylpyruvate; deficient in type II tyrosinemia |
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Term
p-hydroxyphenylpyruvate dioxygenase-deficiency |
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Definition
2. p-hydroxyphenylpyruvate--> homogentisate; deficient in type III tyrosinemia |
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Term
homogentistate 1,2-dioxygenase- deficiency |
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Definition
3. homogentistate-->maleylacetoacetate; deficient in alkaptonuria |
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Term
Maleylacetoacetate isomerase |
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Definition
4. Maleylacetoacetate--> fumarylacetoacetate |
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Term
fumarylacetoacetase- deficiency |
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Definition
5. Fumarylacetoacetate--> acetoacetate; deficiency in type I tyrosinemia |
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Term
3-ketoacyl-CoA transferase |
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Definition
6. acetoacetate--> acetoacetyl-CoA |
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Term
Cause of maple syrup urine disease |
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Definition
branched-chain alpha-ketoacid dehydrogenase |
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