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Lecture

PORPHRYIN METABOLISM

5 Pages
128 Views

Department
Biology
Course Code
BIOLOGY 2D03
Professor
Laura Parker

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Description
PORPHRYIN METABOLISM distribution - heme proteins (hemoglobin, cytochrome) - related proteins (corrin ring, chlorophyll) RDS in heme biosynthesis: succinyl CoA + glycine  ALA explains glycine’s long lifespan in vivo ALAS regulation – tln blocked by IBP Iron present  IBP bound  tln can occur similar strategy for ferritin tln **loss of IBP binding can also destabilize transcripts (transferrin)** regulation: hemin inhibits heme biosynthesis activates globin translation degradation: RBC destruction releases lots of heme heme  biliverdin, bilirubin (yellow) LIVER: bilirubin  bilirupin diglucuoronide  excreted in bile CLINICAL PROBLEMS ACUTE INTERMITTENT PORPHYRIA: most common PBG deaminase activity deficient + ALAS activated  excess PBA/ALA neuropathy, psychosis, abdominal pain PORPHYRIA CUTANEA TARDA uroporhyrinogen decarboxylase deficiency extreme photosensitivity (blistering), hyperpigmentation, facial hair ERYTHROPOETIC PROTOPORPHYRIA deficiency of ferrochelatase photosensitivity, hepatobiliary dysfunction, anemia JAUNDICE – hyper bilirubinemia neonatal – increased destruction of fetal RBC’s UV light treatment hepatocellular – reduced bilirubin conjugation  cannot excrete in bile obstructive – conjugated bilirubin cannot be excreted due to obstruction (pancreatic carcinoma) (gall stones) NUCLEIC ACID METABOLISM Pyrmidines: CUT Purines: GA nucleosides – ribosylated nucle
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