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cytochrome b558 alpha subunit (p22 phagocyte B-cytochrome, neutrophil cytochrome B, 22 kD polypeptide, p21/p22phox, superoxide-generating NADPH oxidase light chain subunit, CYBA)

Function: - alpha subunit of cytochrome b558 - component of the membrane-bound oxidase of phagocytes (NADPH oxidase) that generates superoxide - associates with NOX3 to form a functional NADPH oxidase constitutively generating superoxide - composed of a heavy chain (beta) & a light chain (alpha) - interacts with DUOX1, DUOX2 & TPO - interacts with NOX3 & NOX4 Structure: - belongs to the p22phox family - heme prosthetic group could be coordinated with residues of the light chain, the heavy chain, or both Pathology: - defects in CYBA are a cause of autosomal recessive chronic granulomatous disease

Related

chronic granulomatous disease

General

protein subunit

Properties

SIZE: MW = 21-22 kD MOTIF: transmembrane domain Iron [Fe]-binding site SITE: 93-93 proline-rich region MOTIF: proline residue (SEVERAL)

Database Correlations

OMIM correlations MORBIDMAP 608508 UniProt P13498 Pfam PF05038

References

  1. Rotrosen D, Yeung CL, Leto TL, Malech HL, Kwong CH. Cytochrome b558: the flavin-binding component of the phagocyte NADPH oxidase. Science. 1992 Jun 5;256(5062):1459-62. PMID: 1318579
  2. Segal AW, Abo A. The biochemical basis of the NADPH oxidase of phagocytes. Trends Biochem Sci. 1993 Feb;18(2):43-7. Review. PMID: 8488557
  3. Pawson T Protein modules and signalling networks Nature 373:573 1995 PMID: 7531822
  4. CYBAbase; CYBA mutation db http://bioinf.uta.fi/CYBAbase/
  5. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=CYBA
  6. SHMPD; The Singapore human mutation & polymorphism database http://shmpd.bii.a-star.edu.sg/gene.php?genestart=C&genename=CYBB+%40+GP91-PHOX
  7. UniProt :accession P13498

Component-of

cytochrome b558