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Updated: Dec 26 2018


  • 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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