Updated: 12/26/2018

Peroxisome

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Illustration of peroxisome functions: 1) breakdown of VLFCA. 2) bile acid synthesis.  3) modification of phospholipds. 4) degradation of hydrogen peroxide. 
 
Overview
  • Structure
    • membrane-bound vesicle
    • contain several important enzymes
      • catalase
      • hydrogen-transferring enzymes
      • lipid oxidizing enzymes
        • Refsum disease
          • autosomal recessive 
          • due to mutations in PHYH or PEX7 gene resulting in defects in alpha-oxidation leading to a build up of phytanic acid
          • presentation
            • vision loss
            • anosmia
  • Function
    • degradation of very long chain fatty acids (VLCFAs)
      • > 24 carbons
      • via β-oxidation
      • reduces by 14 carbons and then sends to mitochondria
      • deficient in Zellweger syndrome 
        • build-up of VLCFAs in peroxisomes
        • impaired myelin synthesis
          • severe neurological symptoms
        • hepatomegaly
        • death < 1 year
          • can increase lifespan with diet low in VLCFAs
        • AR
        • other examples of peroxisomal disorders
          • neonatal adrenoleukodystrophy (NALD)
    • bile acid synthesis
      • derived from cholesterol
    • phospholipid modification
      • alter phosphatidylserine and phosphatidylethanolamine
    • degradation of hydrogen peroxide
      • via catalase
      • degrades hydrogen peroxide produced in β-oxidation of fatty acids

 

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