Protein basic information
| LiverAtlas Protein ID |
HuLPr16066 |
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Uniprot ID |
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Uniprot Acc |
P00403;Q37526; |
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Protein name |
Cytochrome c oxidase subunit 2 |
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Comment |
FUNCTION:Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1- 3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1.||COFACTOR:Copper A.||SUBCELLULAR LOCATION:Mitochondrion inner membrane; Multi-pass membrane protein.||DISEASE:Defects in MT-CO2 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Le |
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Subcellular localization |
Mitochondrion inner membrane;Multi-pass membrane protein. |
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Gene name |
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Protein sequence
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MAHAAQVGLQDATSPIMEELITFHDHALMIIFLICFLVLY ALFLTLTTKLTNTNISDAQEMETVWTILPAIILVLIALP SLRILYMTDEVNDPSLTIKSIGHQWYWTYEYTDYGGLIF NSYMLPPLFLEPGDLRLLDVDNRVVLPIEAPIRMMITSQ DVLHSWAVPTLGLKTDAIPGRLNQTTFTATRPGVYYGQC SEICGANHSFMPIVLELIPLKIFEMGPVFTL |
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Database cross reference |
RefSeq Protein accession:YP_003024029
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Liver relevance
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HLPP validation |
Yes/No |
Yes |
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Project name |
Chinese Liver;French Liver;Human Fetal Liver;Human Liver Organelles; |
Ontology annotation
| GO-C |
GO:0016021;C:integral to membrane;IEA:UniProtKB-KW. GO:0005743;C:mitochondrial inner membrane;TAS:Reactome. GO:0070469;C:respiratory chain;IEA:UniProtKB-KW. |
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GO-F |
GO:0005507;F:copper ion binding;IEA:InterPro. GO:0004129;F:cytochrome-c oxidase activity;NAS:UniProtKB. GO:0009055;F:electron carrier activity;IEA:InterPro. GO:0020037;F:heme binding;IEA:InterPro. |
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GO-P |
GO:0006123;P:mitochondrial electron transport, cytochrome c to oxygen;NAS:UniProtKB. |
Post-translational modification
| LiverAtlas Protein ID |
MOD type1 |
Position1 |
Residue1 |
Source name1 |
source ID1 |
Source method |
HLPP validation1 (Yes/no) |
Quality score |
|
HuLPr16066 |
MONO-METHYLATION |
82 |
R |
PhosphoSitePlus |
LTP HTP |
N |
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Pathway
| Pathway name |
