Premium
Biochemistry and molecular biology of chronic granulomatous disease
Author(s) -
Segal A. W.
Publication year - 1992
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01799624
Subject(s) - chronic granulomatous disease , nadph oxidase , biology , vacuole , cytochrome c oxidase , oxidase test , biochemistry , microbiology and biotechnology , enzyme , immunology , cytoplasm
Summary The NADPH oxidase of phagocytic cells is an electron transport chain in the wall of the endocytic vacuole that is activated upon engulfment of the microbe, and is important for its efficient killing and digestion. The oxidase consists of a heterodimeric cytochrome b in the membrane, which is the site of the haem and FAD groups, and two cystolic factors p47‐ phox and p67‐ phox that appear to activate the system. Absence of this oxidase as a result of defects in each of these specialized proteins causes the syndrome of chronic granulomatous disease (CGD), that is characterized by a profound predisposition to pyogenic infection.