Cytochrome b-245 and its involvement in the molecular pathology of chronic granulomatous disease

Hematol Oncol Clin North Am. 1988 Jun;2(2):213-23.

Abstract

Cytochrome b-245 is an integral, and probably the terminal, component of the microbicidal oxidase electron transport chain of phagocytic cells. Current knowledge of the biochemistry and cell and molecular biology of this molecule is described. The molecular basis of chronic granulomatous disease, in which defective electron transport down this chain predisposes to infection and impaired digestion by phagocytes, is explained in terms of anomalies of the cytochrome b and related molecules.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cell Membrane / enzymology
  • Cytochrome b Group / genetics
  • Cytochrome b Group / isolation & purification
  • Cytochrome b Group / metabolism*
  • Cytoplasmic Granules / enzymology
  • Electron Transport
  • Enzyme Activation
  • Female
  • Fishes
  • Genes
  • Genetic Linkage
  • Granulomatous Disease, Chronic / enzymology
  • Granulomatous Disease, Chronic / genetics*
  • Granulomatous Disease, Chronic / immunology
  • Humans
  • Male
  • NADH, NADPH Oxidoreductases / deficiency
  • NADH, NADPH Oxidoreductases / metabolism
  • NADH, NADPH Oxidoreductases / physiology
  • NADP / metabolism
  • NADPH Oxidases
  • Oxidation-Reduction
  • Phagocytes / enzymology
  • Phagocytes / metabolism
  • X Chromosome / analysis

Substances

  • Cytochrome b Group
  • cytochrome b245
  • NADP
  • NADH, NADPH Oxidoreductases
  • NADPH Oxidases