CYC1
entrez
1537
canonical
uniprot
P08574
canonical
ensembl
ENSG00000179091
resolver
ensp
ENSP00000317159
resolver
enst
ENST00000318911
resolver
genesymbol
CYC1
resolver
hgnc
HGNC:2579
resolver
uniprot
Q5U062
resolver
uniprot
Q6FHS7
resolver
uniprot_entry
CY1_HUMAN
resolver
uniprot_sec
Q5U062
resolver
uniprot_sec
Q6FHS7
resolver
amino_acid_sequence
MAAAAASLRGVVLGPRGAGLPGARARGLLCSARPGQLPLRTPQAVALSSKSGLSRGRKVMLSALGMLAAGGAGLAMALHSAVSASDLELHPPSYPWSHRGLLSSLDHTSIRRGFQVYKQVCASCHSMDFVAYRHLVGVCYTEDEAKELAAEVEVQDGPNEDGEMFMRPGKLFDYFPKPYPNSEAARAANNGALPPDLSYIVRARHGGEDYVFSLLTGYCEPPTGVSLREGLYFNPYFPGQAIAMAPPIYTDVLEFDDGTPATMSQIAKDVCTFLRWASEPEHDHRKRMGLKMLMMMALLVPLVYTIKRHKWSVLKSRKLAYRPPK
uniprot
proteins
disease_involvement
DISEASE: Mitochondrial complex III deficiency, nuclear type 6 (MC3DN6) [MIM:615453]: An autosomal recessive disorder caused by mitochondrial dysfunction. It is characterized by onset in early childhood of episodic acute lactic acidosis, ketoacidosis, and insulin-responsive hyperglycemia, usually associated with infection. Laboratory studies show decreased activity of mitochondrial complex III. Psychomotor development is normal. {ECO:0000269|PubMed:23910460}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
7.1.1.8
uniprot
proteins
function
FUNCTION: Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c. Cytochrome c1 is a catalytic core subunit containing a c-type heme. It transfers electrons from the [2Fe-2S] iron-sulfur cluster of the Rieske protein to cytochrome c. {ECO:0000250|UniProtKB:P07143}.
uniprot
proteins
mass_dalton
35422
uniprot
proteins
protein_family
Cytochrome c family
uniprot
proteins
pubmed
10453733
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16421571
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
23910460
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
2536365
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
2836796
uniprot
proteins
pubmed
28844695
uniprot
proteins
pubmed
3036122
uniprot
proteins
sequence_length
325
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250|UniProtKB:P07143}; Single-pass membrane protein {ECO:0000250|UniProtKB:P07143}.
uniprot
proteins
transmembrane_region
TRANSMEM 282..315; /note="Helical"; /evidence="ECO:0000269|PubMed:28844695
uniprot
proteins