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cytochrome b (ubiquinol-cytochrome-c reductase complex cytochrome b subunit, cytochrome b-c1 complex subunit 3, complex 3 subunit 3, MT-CYB, COB, CYTB, MTCYB)
Function:
- component of the ubiquinol-cytochrome c reductase complex (complex 3 or cytochrome b-c1 complex)
Cofactor: binds 2 heme groups non-covalently (putative)
Structure: belongs to the cytochrome b family
Compartment:
- mitochondrial inner membrane
Pathology:
1) defects in MT-CYB are a rare cause of mitochondrial dysfunction underlying different myopathies, including
- mitochondrial encephalomyopathy
- hypertrophic cardiomyopathy
- sporadic mitochondrial myopathy
2) defects in MT-CYB are the cause of histiocytoid cardiomyopathy
3) defects in MT-CYB contribute to Leber hereditary optic neuropathy
Notes:
- Heme 1 (or BL or b562) is low-potential & absorbs at about 562 nm, & heme 2 (or BH or b566) is high-potential & absorbs at about 566 nm (putative)
Also see cytochrome b560 (also referred to as cytochrome B.)
Related
cytochrome B gene
General
cytochrome
mitochondrial membrane protein
transmembrane 8 protein
Properties
SIZE: entity length = 380 aa
MW = 43 kD
COMPARTMENT: mitochondrial inner membrane (GO:0005743)
MOTIF: Iron [Fe]-binding site
SITE: 83-83
Iron [Fe]-binding site
SITE: 97-97
Iron [Fe]-binding site
SITE: 182-182
Iron [Fe]-binding site
SITE: 196-196
Database Correlations
OMIM correlations
UniProt P00156
PFAM correlations
References
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=MT-CYB
- UniProt :accession P00156