UQCC3 (original) (raw)
Ubiquinol-cytochrome c reductase complex assembly factor 3 is a protein that in humans is encoded by the UQCC3 gene. Located in mitochondria, this protein is involved in the assembly of mitochondrial Complex III, stabilizing supercomplexes containing Complex III. Mutations in the UQCC3 gene cause Complex III deficiency with symptoms of hypoglycemia, hypotonia, lactic acidosis, and developmental delays. This protein plays an important role as an antiviral factor, bolstering the ability of cells to inhibit viral replication, independent of interferon production. The UQCC3 protein can be cleaved by OMA1 metalloprotease during mitochondrial depolarization, targeting the cell for apoptosis. Depletion of this protein alters cardiolipin composition, causing cellular and mitochondrial defects.
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| dbo:abstract | Ubiquinol-cytochrome c reductase complex assembly factor 3 is a protein that in humans is encoded by the UQCC3 gene. Located in mitochondria, this protein is involved in the assembly of mitochondrial Complex III, stabilizing supercomplexes containing Complex III. Mutations in the UQCC3 gene cause Complex III deficiency with symptoms of hypoglycemia, hypotonia, lactic acidosis, and developmental delays. This protein plays an important role as an antiviral factor, bolstering the ability of cells to inhibit viral replication, independent of interferon production. The UQCC3 protein can be cleaved by OMA1 metalloprotease during mitochondrial depolarization, targeting the cell for apoptosis. Depletion of this protein alters cardiolipin composition, causing cellular and mitochondrial defects. (en) UQCC3 (англ. Ubiquinol-cytochrome c reductase complex assembly factor 3) – білок, який кодується однойменним геном, розташованим у людей на 11-й хромосомі. Довжина поліпептидного ланцюга білка становить 93 амінокислот, а молекулярна маса — 10 081. Послідовність амінокислот A: АланінD: Аспарагінова кислотаE: Глутамінова кислотаG: ГліцинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин Задіяний у такому біологічному процесі, як синтез АТФ. Локалізований у мембрані, мітохондрії, внутрішній мембрані мітохондрії. (uk) |
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| dbo:wikiPageWikiLink | dbr:Cardiolipin dbr:Protein_secondary_structure dbr:Mutation dbr:Antiviral_protein dbr:Hypoglycemia dbr:Respirasome dbr:Cytochrome_b dbr:Depolarization dbr:Developmental_disability dbr:Innate_immune_system dbr:Signal_peptide dbc:Human_proteins dbr:Gene_expression dbr:OAS3 dbr:Zygosity dbr:Gene dbr:Mitochondrion dbr:Crista dbr:Lactic_acidosis dbr:Apoptosis dbr:Protein_kinase_R dbr:Protein_biosynthesis dbr:Adenosine_triphosphate dbr:Alpha_helix dbr:Amino_acids dbr:Carcinoma dbr:Cell_growth dbr:Cellular_respiration dbr:Chromosome_11 dbr:Protein dbr:Psychomotor_retardation dbr:Regulation_of_gene_expression dbr:Viral_replication dbr:Hypotonia dbr:UQCC2 dbr:Coenzyme_Q_–_cytochrome_c_reductase dbr:Coiled_coil dbr:Tissue_(biology) dbr:Missense_mutation dbr:OMA1 dbc:Mitochondrial_proteins dbr:Interferon dbr:N-terminus dbr:Phosphorylation dbr:Ribonuclease_L dbr:Viral_disease dbr:Protein-protein_interactions dbr:UQCC1 dbr:PHLDA3 |
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| rdf:type | owl:Thing dbo:Biomolecule dbo:Gene wikidata:Q206229 wikidata:Q7187 dbo:HumanGene |
| rdfs:comment | Ubiquinol-cytochrome c reductase complex assembly factor 3 is a protein that in humans is encoded by the UQCC3 gene. Located in mitochondria, this protein is involved in the assembly of mitochondrial Complex III, stabilizing supercomplexes containing Complex III. Mutations in the UQCC3 gene cause Complex III deficiency with symptoms of hypoglycemia, hypotonia, lactic acidosis, and developmental delays. This protein plays an important role as an antiviral factor, bolstering the ability of cells to inhibit viral replication, independent of interferon production. The UQCC3 protein can be cleaved by OMA1 metalloprotease during mitochondrial depolarization, targeting the cell for apoptosis. Depletion of this protein alters cardiolipin composition, causing cellular and mitochondrial defects. (en) UQCC3 (англ. Ubiquinol-cytochrome c reductase complex assembly factor 3) – білок, який кодується однойменним геном, розташованим у людей на 11-й хромосомі. Довжина поліпептидного ланцюга білка становить 93 амінокислот, а молекулярна маса — 10 081. Послідовність амінокислот A: АланінD: Аспарагінова кислотаE: Глутамінова кислотаG: ГліцинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин Задіяний у такому біологічному процесі, як синтез АТФ. Локалізований у мембрані, мітохондрії, внутрішній мембрані мітохондрії. (uk) |
| rdfs:label | UQCC3 (en) UQCC3 (uk) |
| owl:sameAs | wikidata:UQCC3 http://tt.dbpedia.org/resource/UQCC3 dbpedia-uk:UQCC3 https://global.dbpedia.org/id/kQkj |
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