Cytochrome b-c1 complex subunit 8 (UQCRQ)
The protein contains 82 amino acids for an estimated molecular weight of 9906 Da.
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. (updated: Feb. 26, 2020)
Protein identification was indicated in the following studies:
- Hegedűs and co-workers. (2015) Inconsistencies in the red blood cell membrane proteome analysis: generation of a database for research and diagnostic applications. Database (Oxford) 1-8.
- Wilson and co-workers. (2016) Comparison of the Proteome of Adult and Cord Erythroid Cells, and Changes in the Proteome Following Reticulocyte Maturation. Mol Cell Proteomics. 15(6), 1938-1946.
- D'Alessandro and co-workers. (2017) Red blood cell proteomics update: is there more to discover? Blood Transfus. 15(2), 182-187.
Methods
The following articles were analysed to gather the proteome content of erythrocytes.
The gene or protein list provided in the studies were processed using the ID mapping API of Uniprot in September 2018. The number of proteins identified and mapped without ambiguity in these studies is indicated below.
Only Swiss-Prot entries (reviewed) were considered for protein evidence assignation.
| Publication | Identification 1 | Uniprot mapping 2 | Not mapped / Obsolete | TrEMBL | Swiss-Prot |
|---|---|---|---|---|---|
| Goodman (2013) | 2289 (gene list) | 2278 | 53 | 20599 | 2269 |
| Lange (2014) | 1234 | 1234 | 7 | 28 | 1224 |
| Hegedus (2015) | 2638 | 2622 | 0 | 235 | 2387 |
| Wilson (2016) | 1658 | 1528 | 170 | 291 | 1068 |
| d'Alessandro (2017) | 1826 | 1817 | 2 | 0 | 1815 |
| Bryk (2017) | 2090 | 2060 | 10 | 108 | 1942 |
| Chu (2018) | 1853 | 1804 | 55 | 362 | 1387 |
1 as available in the article and/or in supplementary material
2 uniprot mapping returns all protein isoforms as one entry
The compilation of older studies can be retrieved from the Red Blood Cell Collection database.
The data and differentiation stages presented below come from the proteomic study and analysis performed by our partners of the GReX consortium, more details are available in their published work.
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| Variant | Description |
|---|---|
| MC3DN4 |
Biological Process
Cerebellar Purkinje cell layer development
Hippocampus development
Hypothalamus development
Midbrain development
Mitochondrial electron transport, ubiquinol to cytochrome c
Pons development
Pyramidal neuron development
Subthalamus development
Thalamus development
The reference OMIM entry for this protein is 612080
Ubiquinol-cytochrome c reductase, complex iii subunit vii, 9.5-kd; uqcrq
Qpc
DESCRIPTION
Ubiquinone is an essential component of the mitochondrial electron transfer system. UQCRQ is a ubiquinone-binding protein localized to the cytochrome bc1 region of the mitochondrial respiratory chain (Wakabayashi et al., 1985).CLONING
Wakabayashi et al. (1985) cloned bovine Uqcrq, which they called QP-C. The deduced 110-amino acid protein has a relatively hydrophobic and basic N-terminal region, an aspartic acid-rich middle region, and a glutamic acid- and lysine-rich C-terminal region.MAPPING
The International Radiation Hybrid Mapping consortium mapped the UQCRQ gene to chromosome 5 (TMAP REN72017).MOLECULAR GENETICS
In affected members of a large Israeli Bedouin kindred with mitochondrial complex III deficiency nuclear type 4 (MC3DN4; 615159) and a severe neurologic phenotype, Barel et al. (2008) identified a homozygous mutation in the UQCRQ gene (612080.0001). ... More on the omim web site
March 3, 2020: Protein entry updated
Automatic update: Entry updated from uniprot information.
Feb. 23, 2019: Protein entry updated
Automatic update: model status changed
Nov. 17, 2018: Protein entry updated
Automatic update: OMIM entry 612080 was added.
Oct. 19, 2018: Additional information
Initial protein addition to the database. This entry was referenced in Bryk and co-workers. (2017).