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GeneQ18032298· pop 5· linked from 13 articles

Also known as MC3DN5, QCR2, UQCR2, ubiquinol-cytochrome c reductase core protein II, ubiquinol-cytochrome c reductase core protein 2

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 (UQCR, Complex III or Cytochrome bc1 complex), 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", a

Gene data

UQCRC2
Name
ubiquinol-cytochrome c reductase core protein 2
Type
protein-coding
Position
21,952,636–21,990,814 (+)
Aliases
MC3DN5, QCR2, UQCR2
RefSeq RNA
NM_003366.4
RefSeq protein
NP_003357.2

The protein encoded by this gene is located in the mitochondrion, where it is part of the ubiquinol-cytochrome c reductase complex (also known as complex III). This complex constitutes a part of the mitochondrial respiratory chain. Defects in this gene are a cause of mitochondrial complex III deficiency nuclear type 5. [provided by RefSeq, Jul 2015].

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Gene · Ensembl

ubiquinol-cytochrome c reductase core protein 2

Symbol
UQCRC2
Biotype
Protein coding
Organism
Homo sapiens
Location
16:21,952,636-21,990,814
Strand
Forward (+)
Assembly
GRCh38
View on Ensembl →

via Ensembl · EMBL-EBI

Wikidata facts

Show 5 more facts
HomoloGene ID
37764
genomic start
21953288
genomic end
21994981
cytogenetic location
16p12.2
Sources (4)

via Wikidata · CC0

~3 min read

Article

6 sections
Contents
  • Structure
  • Function
  • Clinical Significance
  • Interactions
  • References
  • Further reading

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 (UQCR, Complex III or Cytochrome bc1 complex), 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." Defects in UQCRC2 are associated with mitochondrial complex III deficiency, nuclear, type 5.

== Structure == UQCRC2 is located on the p arm of chromosome 16 in position 12.2 and has 14 exons. The UQCRC2 gene produces a 48.4 kDa protein composed of 453 amino acids. UQCRC2 belongs to the peptidase M16 family and UQCRC2/QCR2 subfamily. UQCRC2 has a transit peptide domain. Ubiquinol Cytochrome c Reductase (b-c1 complex) contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1). UQCRC2 is part of the hydrophobic core of the b-c1 complex and is necessary for the stabilization of Ubiquinol Cytochrome c Reductase.

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