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IPR017276

Source: InterPro

Description

IPR017276 is a Synthesis of cytochrome c oxidase, Sco1/Sco2.

<p>This entry represents cytochrome c oxidase assembly factors Sco1 and Sco2 (Synthesis of Cytochrome c Oxidase, factors 1 and 2), mitochondrial inner membrane-tethered metallochaperones that have regulatory roles in the maintenance of cellular copper homeostasis. These proteins are essential for the assembly of the catalytic core of cytochrome c oxidase (COX or complex IV), as well as other roles in copper homeostasis such as mitochondrial redox signalling [[cite:PUB00042747]]. Both Sco1 and Sco2 contain highly conserved CXXXC motifs thought to be required for copper binidng.</p> <p>COX is the terminal enzyme of the energy transducing respiratory chain in eukaryotes and certain prokaryotes. It catalyses the transfer of electrons from cytochrome c to molecular oxygen and pumps protons across the mitochondrial inner membrane to establish a proton gradient for ATP synthesis. It consists of 12-13 protein subunits, with 3 subunits (Cox1-Cox3) forming the enzyme core. COX uses haem and copper as cofactors: Cox1 contains a 1-copper centre (CuB) that interacts with the haem moiety and Cox2 contains a 2-copper centre (CuA). Sco1 and Sco2 act as copper chaperones, transporting copper to the CuA site in Cox2, and are thought to have cooperative functions in COX assembly [[cite:PUB00032292], [cite:PUB00042748]]. In addition, human Sco2 is also the downstream mediator of the balance between the utilization of respiratory and glycolytic pathways [[cite:PUB00042749]] and both Sco1 and Sco2 may have regulatory roles in regulating cellular copper levels (homeostasis) [[cite:PUB00042747]]. Sco2 may have a copper-level-detection signalling role, acting upstream and in conjunction with Sco1.</p> <p>Defects in Sco1 are a cause of cytochrome c oxidase deficiency (COX deficiency) [OMIM:220110], a clinically heterogeneous disorder with features ranging from isolated myopathy to severe multisystem disease, and onset from infancy to adulthood. Defects in Sco2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) [OMIM:604377] [OMIM:220110], which is characterised by hypertrophic cardiomyopathy, lactic acidosis, and gliosis.</p>

This description is obtained from EB-eye REST.

Associated GO terms

GO predictions are based solely on the InterPro-to-GO mappings published by EMBL-EBI, which are in turn based on the mapping of predicted domains to the InterPro dataset. The InterPro-to-GO mapping was last updated on , while the GO metadata was last updated on .

GO term Namespace Name Definition Relationships
GO:0005507
Molecular function Copper ion binding Interacting selectively and non-covalently with copper (Cu) ions.
Subterm of
GO:0005743
Cellular component Mitochondrial inner membrane The inner, i.e. lumen-facing, lipid bilayer of the mitochondrial envelope. It is highly folded to form cristae.
Subterm of
GO:0006825
Biological process Copper ion transport The directed movement of copper (Cu) ions into, out of or within a cell, or between cells, by means of some agent such as a transporter or pore.
Subterm of
GO:0006878
Biological process Cellular copper ion homeostasis Any process involved in the maintenance of an internal steady state of copper ions at the level of a cell.
Subterm of
GO:0008535
Biological process Respiratory chain complex IV assembly The aggregation, arrangement and bonding together of a set of components to form respiratory chain complex IV (also known as cytochrome c oxidase), the terminal member of the respiratory chain of the mitochondrion and some aerobic bacteria. Cytochrome c oxidases are multi-subunit enzymes containing from 13 subunits in the mammalian mitochondrial form to 3-4 subunits in the bacterial forms.
Subterm of

Associated Lotus transcripts 4

Transcript Name Description Predicted domains Domain count
Lj1g3v0114190.2
– PREDICTED: protein SCO1 homolog 1, mitochondrial-like [Cicer arietinum] gi|502104167|ref|XP_004492460.1|
Domains
17
Lj1g3v2372130.2
– PREDICTED: protein SCO1 homolog 1, mitochondrial-like [Cicer arietinum] gi|502104167|ref|XP_004492460.1|
Domains
17
LotjaGi1g1v0443200.1
– Protein SCO1, mitochondrial-like protein; TAIR: AT3G08950.1 electron transport SCO1/SenC family protein; Swiss-Prot: sp|Q8VYP0|SCO11_ARATH Protein SCO1 homolog 1, mitochondrial; TrEMBL-Plants: tr|A0A0B2QKB0|A0A0B2QKB0_GLYSO Protein SCO1 like 1, mitochondrial; Found in the gene: LotjaGi1g1v0443200
Domains
17
LotjaGi2g1v0453900.2
– Protein SCO1, mitochondrial-like protein; TAIR: AT3G08950.1 electron transport SCO1/SenC family protein; Swiss-Prot: sp|Q8VYP0|SCO11_ARATH Protein SCO1 homolog 1, mitochondrial; TrEMBL-Plants: tr|A0A0B2QKB0|A0A0B2QKB0_GLYSO Protein SCO1 like 1, mitochondrial; Found in the gene: LotjaGi2g1v0453900
Domains
17

Co-occuring domains 1

A list of co-occurring predicted domains within the L. japonicus gene space:

Predicted domain Source Observations Saturation (%)
mobidb-lite MobiDBLite 1 25.00