Cytochrome c oxidase subunit I (original) (raw)

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Cytochrome c oxidase I (COX1) also known as mitochondrially encoded cytochrome c oxidase I (MT-CO1) is a protein that in humans is encoded by the MT-CO1 gene. In other eukaryotes, the gene is called COX1, CO1, or COI. Cytochrome c oxidase I is the main subunit of the cytochrome c oxidase complex. Mutations in MT-CO1 have been associated with Leber's hereditary optic neuropathy (LHON), acquired idiopathic sideroblastic anemia, Complex IV deficiency, colorectal cancer, sensorineural deafness, and recurrent myoglobinuria.

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dbo:abstract Cytochrome c oxidase I (COX1) also known as mitochondrially encoded cytochrome c oxidase I (MT-CO1) is a protein that in humans is encoded by the MT-CO1 gene. In other eukaryotes, the gene is called COX1, CO1, or COI. Cytochrome c oxidase I is the main subunit of the cytochrome c oxidase complex. Mutations in MT-CO1 have been associated with Leber's hereditary optic neuropathy (LHON), acquired idiopathic sideroblastic anemia, Complex IV deficiency, colorectal cancer, sensorineural deafness, and recurrent myoglobinuria. (en) Het CO1-gen, MT-CO1-gen of cytochroom c oxidase subunit 1-gen is een stofwisselingsgen dat voorkomt op het mitochondriaal DNA van alle levende wezens. Het gen heeft een lengte van 648 basenparen. Het gen codeert voor de subunit 1 van cytochroom c oxidase (COX1), een enzym dat een cruciale rol speelt in de energiehuishouding van het organisme, met name in de oxidatieve fosforylering. Mitochondriaal DNA heeft een hoge mutatiesnelheid, waardoor de nucleotidesequentie (de juiste volgorde van de verschillende basenparen) tussen verschillende soorten sterk varieert. Daarenboven heeft het CO1-gen eigenschappen die het bij uitstek geschikt maken voor DNA-barcoding, althans bij dieren: * het gen is kort genoeg om de nucleotidesequentie met de huidige technologie snel te kunnen bepalen; * het gen is lang genoeg om voldoende variatie in de nucleotidesequentie te vertonen over alle soorten heen, zodat twee verschillende soorten duidelijk kunnen onderscheiden worden; zo verschillen twee nauw verwante soorten als de mens en de chimpansee op ongeveer 60 plaatsen; * daarentegen vertoont het binnen één soort een zeer beperkte variatie; zo verschillen bij de mens maximaal twee van de 648 basenparen; Bij planten is het CO1-gen minder geschikt voor DNA-barcoding door de veel lagere mutatiesnelheid, en moeten andere genen gezocht worden. (nl) COX1 (англ. Cytochrome c oxidase subunit I) – білок, який кодується однойменним геном, який у людей є частиною мітохондріальної ДНК. Довжина поліпептидного ланцюга білка становить 513 амінокислот, а молекулярна маса — 57 041. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин Кодований геном білок за функцією належить до оксидоредуктаз. Задіяний у таких біологічних процесах, як транспорт, транспорт електронів, дихальний ланцюг. Білок має сайт для зв'язування з іоном міді, іонами металів, іоном заліза, гемом. Локалізований у мембрані, мітохондрії, внутрішній мембрані мітохондрії. (uk)
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dbp:caption Structure of the 13-subunit oxidized cytochrome c oxidase. (en)
dbp:cdd cd01663 (en)
dbp:interpro IPR000883 (en)
dbp:name Cytochrome c oxidase subunit I (en)
dbp:opmFamily 4 (xsd:integer)
dbp:opmProtein 1 (xsd:integer)
dbp:pfam PF00115 (en)
dbp:prosite PDOC00074 (en)
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dbp:symbol COX1 or COI (en)
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rdfs:comment Cytochrome c oxidase I (COX1) also known as mitochondrially encoded cytochrome c oxidase I (MT-CO1) is a protein that in humans is encoded by the MT-CO1 gene. In other eukaryotes, the gene is called COX1, CO1, or COI. Cytochrome c oxidase I is the main subunit of the cytochrome c oxidase complex. Mutations in MT-CO1 have been associated with Leber's hereditary optic neuropathy (LHON), acquired idiopathic sideroblastic anemia, Complex IV deficiency, colorectal cancer, sensorineural deafness, and recurrent myoglobinuria. (en) Het CO1-gen, MT-CO1-gen of cytochroom c oxidase subunit 1-gen is een stofwisselingsgen dat voorkomt op het mitochondriaal DNA van alle levende wezens. Het gen heeft een lengte van 648 basenparen. Het gen codeert voor de subunit 1 van cytochroom c oxidase (COX1), een enzym dat een cruciale rol speelt in de energiehuishouding van het organisme, met name in de oxidatieve fosforylering. Bij planten is het CO1-gen minder geschikt voor DNA-barcoding door de veel lagere mutatiesnelheid, en moeten andere genen gezocht worden. (nl) COX1 (англ. Cytochrome c oxidase subunit I) – білок, який кодується однойменним геном, який у людей є частиною мітохондріальної ДНК. Довжина поліпептидного ланцюга білка становить 513 амінокислот, а молекулярна маса — 57 041. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин (uk)
rdfs:label Cytochrome c oxidase subunit I (en) CO1 (nl) COX1 (uk)
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