Defective peroxisomal cleavage of the C27-steroid side chain in the cerebro-hepato-renal syndrome of Zellweger.

Lignende værker

• Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.
  af: Singh, I, et al.
  Udgivet: (1984)
• Tapetoretinal degeneration in the cerebro-hepato-renal (Zellweger's) syndrome.
  af: Garner, A., et al.
  Udgivet: (1982)
• In vivo and vitro studies on formation of bile acids in patients with Zellweger syndrome. Evidence that peroxisomes are of importance in the normal biosynthesis of both cholic and chenodeoxycholic acid.
  af: Kase, B F, et al.
  Udgivet: (1985)
• Biosynthesis of bile acids in man. Hydroxylation of the C27-steroid side chain.
  af: Björkhem, I, et al.
  Udgivet: (1975)
• Metabolism of prostaglandin F2 alpha in Zellweger syndrome. Peroxisomal beta-oxidation is a major importance for in vivo degradation of prostaglandins in humans.
  af: Diczfalusy, U, et al.
  Udgivet: (1991)