UQCRH

Last updated • a couple of secsFrom Wikipedia, The Free Encyclopedia
UQCRH
Protein UQCRH PDB 1bcc.png
Identifiers
Aliases UQCRH , QCR6, UQCR8, ubiquinol-cytochrome c reductase hinge protein
External IDs OMIM: 613844 MGI: 3641869 HomoloGene: 103848 GeneCards: UQCRH
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_006004
NM_001297565
NM_001297566

n/a

RefSeq (protein)

NP_001284494
NP_001284495
NP_005995

n/a

Location (UCSC) Chr 1: 46.3 – 46.32 Mb n/a
PubMed search [2] [3]
Wikidata
View/Edit Human View/Edit Mouse

Cytochrome b-c1 complex subunit 6, mitochondrial is a protein that in humans is encoded by the UQCRH gene. [4] [5]


Its gene product is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase (UQCR, Complex III or Cytochrome bc1 complex; E.C. 1.10.2.2), which consists of the products of one mitochondrially encoded gene, MTCYTB (mitochondrial cytochrome b) and ten nuclear genes: UQCRC1, UQCRC2, Cytochrome c1, UQCRFS1 (Rieske protein), UQCRB, "11kDa protein", UQCRH (cyt c1 Hinge protein), Rieske Protein presequence, "cyt. c1 associated protein", and "Rieske-associated protein".

Related Research Articles

<span class="mw-page-title-main">Coenzyme Q – cytochrome c reductase</span> Class of enzymes

The coenzyme Q : cytochrome c – oxidoreductase, sometimes called the cytochrome bc1 complex, and at other times complex III, is the third complex in the electron transport chain, playing a critical role in biochemical generation of ATP. Complex III is a multisubunit transmembrane protein encoded by both the mitochondrial and the nuclear genomes. Complex III is present in the mitochondria of all animals and all aerobic eukaryotes and the inner membranes of most eubacteria. Mutations in Complex III cause exercise intolerance as well as multisystem disorders. The bc1 complex contains 11 subunits, 3 respiratory subunits, 2 core proteins and 6 low-molecular weight proteins.

<span class="mw-page-title-main">Rieske protein</span> Protein family with an iron–sulfur center transferring electrons

Rieske proteins are iron–sulfur protein (ISP) components of cytochrome bc1 complexes and cytochrome b6f complexes and are responsible for electron transfer in some biological systems. John S. Rieske and co-workers first discovered the protein and in 1964 isolated an acetylated form of the bovine mitochondrial protein. In 1979 Trumpower's lab isolated the "oxidation factor" from bovine mitochondria and showed it was a reconstitutively-active form of the Rieske iron-sulfur protein
It is a unique [2Fe-2S] cluster in that one of the two Fe atoms is coordinated by two histidine residues rather than two cysteine residues. They have since been found in plants, animals, and bacteria with widely ranging electron reduction potentials from -150 to +400 mV.

<span class="mw-page-title-main">Cytochrome b</span> Mitochondrial protein involved in the respiratory chain

Cytochrome b within both molecular and cell biology, is a protein found in the mitochondria of eukaryotic cells. It functions as part of the electron transport chain and is the main subunit of transmembrane cytochrome bc1 and b6f complexes.

<span class="mw-page-title-main">MT-CYB</span> A mitochondrial protein-coding gene whose product is involved in the respiratory chain

Cytochrome b is a protein that in humans is encoded by the MT-CYB gene. Its gene product is a subunit of the respiratory chain protein ubiquinol–cytochrome c reductase, which consists of the products of one mitochondrially encoded gene, MT-CYB, and ten nuclear genes—UQCRC1, UQCRC2, CYC1, UQCRFS1, UQCRB, "11kDa protein", UQCRH, Rieske protein presequence, "cyt c1 associated protein", and Rieske-associated protein.

<span class="mw-page-title-main">CYC1</span> Protein-coding gene in the species Homo sapiens

Cytochrome c1, heme protein, mitochondrial (CYC1), also known as UQCR4, MC3DN6, Complex III subunit 4, Cytochrome b-c1 complex subunit 4, or Ubiquinol-cytochrome-c reductase complex cytochrome c1 subunit is a protein that in humans is encoded by the CYC1 gene. CYC1 is a respiratory subunit of Ubiquinol Cytochrome c Reductase, which is located in the inner mitochondrial membrane and is part of the electron transport chain. Mutations in this gene may cause mitochondrial complex III deficiency, nuclear, type 6.

<span class="mw-page-title-main">UQCRC1</span> Protein-coding gene in the species Homo sapiens

Cytochrome b-c1 complex subunit 1, mitochondrial is a protein that in humans is encoded by the UQCRC1 gene.

<span class="mw-page-title-main">COX17</span> Protein-coding gene in the species Homo sapiens

Cytochrome c oxidase copper chaperone is a protein that in humans is encoded by the COX17 gene.

<span class="mw-page-title-main">BCS1L</span> Protein-coding gene in the species Homo sapiens

Mitochondrial chaperone BCS1 (BCS1L), also known as BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone (h-BCS1), is a protein that in humans is encoded by the BCS1L gene. BCS1L is a chaperone protein involved in the assembly of Ubiquinol Cytochrome c Reductase, which is located in the inner mitochondrial membrane and is part of the electron transport chain. Mutations in this gene are associated with mitochondrial complex III deficiency, GRACILE syndrome, and Bjoernstad syndrome.

<span class="mw-page-title-main">COX6B1</span> Protein-coding gene in the species Homo sapiens

Cytochrome c oxidase subunit 6B1 is an enzyme that in humans is encoded by the COX6B1 gene. Cytochrome c oxidase 6B1 is a subunit of the cytochrome c oxidase complex, also known as Complex IV, the last enzyme in the mitochondrial electron transport chain. Mutations of the COX6B1 gene are associated with severe infantile encephalomyopathy and mitochondrial complex IV deficiency (MT-C4D).

<span class="mw-page-title-main">UCRC</span> Protein-coding gene in the species Homo sapiens

Ubiquinol-cytochrome c reductase complex , also known as UCRC or UQCR10, is a human gene.

<span class="mw-page-title-main">COX7A1</span> Protein-coding gene in the species Homo sapiens

Cytochrome c oxidase polypeptide 7A1, mitochondrial is an enzyme that in humans is encoded by the COX7A1 gene.

<span class="mw-page-title-main">UQCRB</span> Protein

Ubiquinol-cytochrome c reductase binding protein, also known as UQCRB, Complex III subunit 7, QP-C, or Ubiquinol-cytochrome c reductase complex 14 kDa protein is a protein which in humans is encoded by the UQCRB gene. This gene encodes a subunit of the ubiquinol-cytochrome c oxidoreductase complex, which consists of one mitochondrial-encoded and 10 nuclear-encoded subunits. Mutations in this gene are associated with mitochondrial complex III deficiency. Alternatively spliced transcript variants have been found for this gene. Related pseudogenes have been identified on chromosomes 1, 5 and X.

<span class="mw-page-title-main">UQCRC2</span> Protein-coding gene in the species Homo sapiens

Cytochrome b-c1 complex subunit 2, mitochondrial (UQCRC2), also known as QCR2, UQCR2, or MC3DN5 is a protein that in humans is encoded by the UQCRC2 gene. The product of UQCRC2 is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase, which consists of the products of one mitochondrially encoded gene, MTCYTB and ten nuclear genes: UQCRC1, UQCRC2, Cytochrome c1, UQCRFS1, UQCRB, "11kDa protein", UQCRH, Rieske Protein presequence, "cyt. c1 associated protein", and "Rieske-associated protein." Defects in UQCRC2 are associated with mitochondrial complex III deficiency, nuclear, type 5.

<span class="mw-page-title-main">UQCRFS1</span> Protein-coding gene in the species Homo sapiens

Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1, also known as UQCRFS1, Rieske iron-sulfur (Fe-S) protein, Cytochrome b-c1 complex subunit 5, or Complex III subunit 5 is a protein which in humans is encoded by the UQCRFS1 gene. UQCRFS1 is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase, which consists of the products of one mitochondrially encoded gene, MTCYTB and ten nuclear genes UQCRC1, UQCRC2, Cytochrome C1, UQCRFS1, UQCRB,UQCRQ, UQCRH, UCRC, and UQCR.

<span class="mw-page-title-main">COX6C</span> Protein-coding gene in the species Homo sapiens

Cytochrome c oxidase subunit 6C is an enzyme that in humans is encoded by the COX6C gene.

<span class="mw-page-title-main">COX7C</span> Protein-coding gene in the species Homo sapiens

Cytochrome c oxidase subunit 7C, mitochondrial is an enzyme that in humans is encoded by the COX7C gene.

<span class="mw-page-title-main">UQCR11</span> Protein-coding gene in the species Homo sapiens

UQCR11 is a protein that in humans is encoded by the UQCR11 gene. UQCR11 is the smallest known component of Complex III in the mitochondrial respiratory chain.

<span class="mw-page-title-main">UQCRQ</span> Protein-coding gene in the species Homo sapiens

Ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa is a protein that in humans is encoded by the UQCRQ gene. This ubiqinone-binding protein is a subunit of mitochondrial Complex III in the electron transport chain. A mutation in the UQCRQ gene has been shown to cause severe neurological disorders. Infection by Trypanosoma cruzi can cause oxidative modification of this protein in cardiac muscle tissue.

Tetratricopeptide repeat domain 19, also known as TPR repeat protein 19 or Tetratricopeptide repeat protein 19, mitochondrial is a protein that in humans is encoded by the TTC19 gene. This gene encodes a protein with a tetratricopeptide repeat (TPR) domain containing several TPRs of about 34 amino acids each. These repeats are found in a variety of organisms including bacteria, fungi and plants, and are involved in a variety of functions including protein-protein interactions. This protein is embedded in the inner mitochondrial membrane and is involved in the formation of the mitochondrial respiratory chain III. It has also been suggested that this protein plays a role in cytokinesis. Mutations in this gene cause mitochondrial complex III deficiency. Alternatively spliced transcript variants have been found for this gene.

LYR motif containing 7, also known as Complex III assembly factor LYRM7 or LYR motif-containing protein 7 is a protein that in humans is encoded by the LYRM7 gene. The protein encoded by this gene is a nuclear-encoded mitochondrial matrix protein that stabilizes UQCRFS1 and chaperones it to the CIII complex. Defects in this gene are a cause of mitochondrial complex III deficiency, nuclear type 8. Three transcript variants encoding two different isoforms have been found for this gene.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000173660 - Ensembl, May 2017
  2. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  3. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. Ohta S, Goto K, Arai H, Kagawa Y (Jan 1988). "An extremely acidic amino-terminal presequence of the precursor for the human mitochondrial hinge protein". FEBS Lett. 226 (1): 171–5. doi: 10.1016/0014-5793(87)80573-2 . PMID   2826252.
  5. "Entrez Gene: UQCRH ubiquinol-cytochrome c reductase hinge protein".

Further reading