Cytochrome b-c1 complex subunit 8

NameCytochrome b-c1 complex subunit 8
Synonyms
  • Complex III subunit 8
  • Complex III subunit VIII
  • Ubiquinol-cytochrome c reductase complex 9.5 kDa protein
  • Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C
Gene NameUQCRQ
OrganismHuman
Amino acid sequence
>lcl|BSEQ0017247|Cytochrome b-c1 complex subunit 8
MGREFGNLTRMRHVISYSLSPFEQRAYPHVFTKGIPNVLRRIRESFFRVVPQFVVFYLIY
TWGTEEFERSKRKNPAAYENDK
Number of residues82
Molecular Weight9906.315
Theoretical pI10.5
GO Classification
Functions
  • ubiquinol-cytochrome-c reductase activity
Processes
  • small molecule metabolic process
  • cellular metabolic process
  • respiratory electron transport chain
  • hippocampus development
  • pyramidal neuron development
  • thalamus development
  • cerebellar Purkinje cell layer development
  • hypothalamus development
  • midbrain development
  • pons development
  • subthalamus development
Components
  • respiratory chain
  • mitochondrion
  • mitochondrial inner membrane
General FunctionUbiquinol-cytochrome-c reductase activity
Specific FunctionThis is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit, together with cytochrome b, binds to ubiquinone.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID2605590
UniProtKB IDO14949
UniProtKB Entry NameQCR8_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0017248|Cytochrome b-c1 complex subunit 8 (UQCRQ)
ATGGGCCGCGAGTTTGGGAATCTGACGCGGATGCGGCATGTGATCAGCTACAGCTTGTCA
CCGTTCGAGCAGCGCGCCTATCCGCACGTCTTCACTAAAGGAATCCCCAATGTTCTGCGC
CGCATTCGGGAGTCTTTCTTTCGCGTGGTGCCGCAGTTTGTAGTGTTTTATCTTATCTAC
ACATGGGGGACTGAAGAGTTCGAGAGATCCAAGAGGAAGAATCCAGCTGCCTATGAAAAT
GACAAATGA
GenBank Gene IDD50369
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:29594
Chromosome Location5
Locus5q31.1
References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. 15489334
  2. Schagger H, Brandt U, Gencic S, von Jagow G: Ubiquinol-cytochrome-c reductase from human and bovine mitochondria. Methods Enzymol. 1995;260:82-96. 8592474
  3. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. 21269460
  4. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. 25944712
  5. Barel O, Shorer Z, Flusser H, Ofir R, Narkis G, Finer G, Shalev H, Nasasra A, Saada A, Birk OS: Mitochondrial complex III deficiency associated with a homozygous mutation in UQCRQ. Am J Hum Genet. 2008 May;82(5):1211-6. doi: 10.1016/j.ajhg.2008.03.020. Epub 2008 Apr 24. 18439546