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8 Cards in this Set
- Front
- Back
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purpose
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VLCFA need primary Beta oxidation inside the peroxisome to shorted the chain
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what happens after peroxisome
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VLCFA transported to the mitochondria for normal B-oxidation
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difference between peroxisome and regular oxidation
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initial dehydrogenase
FAD-containing acyl Coa oxidase |
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what does the peroxisome enzyme do
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FAD containing acyl Coa oxidase
FAD --> FADH2 at the same time O2 --> H202 |
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second step of peroxisome oxidation
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H202 --> H20 via catalase
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Zwellweger
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cerebrohepatorenal syndrome
defect in peroxisome biogenesis in all tissues |
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adrenoleukodystrophy
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defect in peroxisomal activation of VLCFA
leads to the accumulation of VLCFA in blood and tissues X-linked |
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peroxisomal B-oxidation deficincies
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Zwellweger (per. biogenesis in all tissue
adrenoleukodytrophy (per. activation of VLCFA) |
