Q99KD6 · CO72R_MOUSE

  • Protein
    Cytochrome c oxidase subunit 7A2-like, mitochondrial
  • Gene
    Cox7a2l
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Assembly factor that mediates the formation of some mitochondrial respiratory supercomplexes (respirasomes), thereby promoting oxidative phosphorylation and energy metabolism (PubMed:23812712, PubMed:23857330, PubMed:25470551, PubMed:27545886, PubMed:27775717, PubMed:27997587, PubMed:32637615, PubMed:34616041, PubMed:38575788).
Acts as a molecular adapter that associates with both mitochondrial respiratory complexes III (CIII) and IV (CIV), promoting their association (PubMed:27545886, PubMed:27775717, PubMed:32637615, PubMed:34616041, PubMed:38575788).
Mediates the formation of various mitochondrial respiratory supercomplexes, such as MCIII2IV2, composed of two CIII and two CIV, and the CS-respirasome (MCI1III2IV2), composed of one CI, two CIII and two CIV (PubMed:27775717, PubMed:32637615, PubMed:34616041, PubMed:38575788).
Not involved in the formation of the canonical respirasome (MCI1III2IV1), composed of one CI, two CIII and one CIV (PubMed:38575788).
The formation of different respirasomes is important for cell adaptation to oxygen conditions and prevent metabolic exhaustion: supercomplexes mediated by COX7A2L/SCAF1 are required to maintain oxidative phosphorylation upon low oxygen conditions and promote metabolic rewiring toward glycolysis (PubMed:38575788).

Pathway

Energy metabolism; oxidative phosphorylation.

GO annotations

AspectTerm
Cellular Componentmitochondrial inner membrane
Cellular Componentrespiratory chain complex IV
Biological Processmitochondrial electron transport, cytochrome c to oxygen

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    Cytochrome c oxidase subunit 7A2-like, mitochondrial
  • Alternative names
    • Supercomplex assembly factor 1

Gene names

    • Name
      Cox7a2l
    • Synonyms
      Scaf1
      , Scafi

Organism names

  • Taxonomic identifier
  • Strains
    • 129
    • FVB/N
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus

Accessions

  • Primary accession
    Q99KD6

Organism-specific databases

Subcellular Location

Features

Showing features for transmembrane.

TypeIDPosition(s)Description
Transmembrane81-106Helical

Keywords

Phenotypes & Variants

Disruption phenotype

Knockout homozygous mice manifest decreased mitochondrial respiratory activities and impaired formation of mitochondrial supercomplexes in muscles (PubMed:23857330).
Mice display muscle weakness and exhibit heat production failure in the cold (PubMed:23857330).

Features

Showing features for mutagenesis.

TypeIDPosition(s)Description
Mutagenesis75Abolished ability to promote association between both mitochondrial respiratory complexes III (CIII) and IV (CIV).

PTM/Processing

Features

Showing features for transit peptide, chain, modified residue.

Type
IDPosition(s)Description
Transit peptide1-54Mitochondrion
ChainPRO_000046146155-113Cytochrome c oxidase subunit 7A2-like, mitochondrial
Modified residue68N6-acetyllysine

Keywords

Expression

Induction

Specifically induced in muscle upon exercise (PubMed:36253618).
Expression is induced by ATF4 downstream of the EIF2AK3/PERK-mediated unfolded protein response, thereby increasing formation of respiratory chain supercomplexes (PubMed:31023583).

Interaction

Subunit

Interacts with the mitochondrial respiratory complexes III (CIII) and IV (CIV), promoting their association.

Family & Domains

Sequence similarities

Belongs to the cytochrome c oxidase VIIa family.

Keywords

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Sequence processing
    The displayed sequence is further processed into a mature form.
  • Length
    113
  • Mass (Da)
    12,562
  • Last updated
    2001-06-01 v1
  • MD5 Checksum
    BE37C3A8914C7EA9F0A92160B327D9C4
MYYKFSSFTQKLAGAWASEAYTPQGLKPVSTEAPPIIFATPTKLTSSVTAYDYSGKNKVPELQKFFQKADGVPIHLKRGLPDQMLYRTTMALTLGGTIYCLIALYMASQPRNK

Polymorphism

There are two alleles for Cox7a2l/Scaf1 depending on mouse strains; a 113 amino-acid long protein, which corresponds to a functional protein (this protein), and a 111 amino-acid long protein caused by a two-amino acid deletion (AC Q61387) (PubMed:23812712, PubMed:36253618).
Only the 113 amino-acid form (this proteins) can induce the mitochondrial respiratory supercomplex assembly by promoting interaction between complex III (CIII) and complex IV (CIV) (PubMed:23812712, PubMed:36253618).

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
BC004715
EMBL· GenBank· DDBJ
AAH04715.1
EMBL· GenBank· DDBJ
mRNA
BC085296
EMBL· GenBank· DDBJ
AAH85296.1
EMBL· GenBank· DDBJ
mRNA

Similar Proteins

Disclaimer

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