Anti-MTCO1 Antibody (CAB17889)
- SKU:
- CAB17889
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-MTCO1 Antibody |
抗体コード: | CAB17889 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant protein of human MTCO1. |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | HeLa, HepG2, 293T, THP-1 |
免疫原: | Recombinant protein of human MTCO1. |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | Email for sequence |
遺伝子ID: | 4512 |
Uniprot: | P00395 |
セルラーロケーション: | |
計算された分子量: | 57kDa |
観察された分子量: | 40kDa |
同義語: | COI, MTCO1, COX1, MT-CO1 |
バックグラウンド: |
UniProt Protein Function: | COX1: 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. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B. Defects in MT-CO1 are a cause of Leber hereditary optic neuropathy (LHON). LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. MT-CO1 may play a role in the pathogenesis of acquired idiopathic sideroblastic anemia, a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria. Mitochondrial iron overload may be attributable to mutations of mitochondrial DNA because these can cause respiratory chain dysfunction, thereby impairing reduction of ferric iron to ferrous iron. The reduced form of iron is essential to the last step of mitochondrial heme biosynthesis. Defects in MT-CO1 are a cause of mitochondrial complex IV deficiency (MT-C4D); 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 Leigh syndrome. Defects in MT-CO1 are associated with recurrent myoglobinuria mitochondrial (RM-MT). Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Defects in MT-CO1 are a cause of deafness sensorineural mitochondrial (DFNM). DFNM is a form of non-syndromic deafness with maternal inheritance. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies. Defects in MT-CO1 are a cause of colorectal cancer (CRC). Belongs to the heme-copper respiratory oxidase family. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Energy Metabolism - oxidative phosphorylation; Oxidoreductase; Membrane protein, multi-pass; EC 1.9.3.1; Mitochondrial Chromosomal Location of Human Ortholog: - Disease: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes; Deafness, Nonsyndromic Sensorineural, Mitochondrial |
UniProt Code: | P00395 |
NCBI GenInfo Identifier: | 116977 |
NCBI Gene ID: | 4512 |
NCBI Accession: | P00395.1 |
UniProt Related Accession: | P00395 |
Molecular Weight: | |
NCBI Full Name: | Cytochrome c oxidase subunit 1 |
NCBI Synonym Full Names: | mitochondrially encoded cytochrome c oxidase I |
NCBI Official Symbol: | MT-CO1 |
NCBI Official Synonym Symbols: | COI; MTCO1; COX1 |
NCBI Protein Information: | cytochrome c oxidase subunit I |
UniProt Protein Name: | Cytochrome c oxidase subunit 1 |
UniProt Synonym Protein Names: | Cytochrome c oxidase polypeptide I |
Protein Family: | Cox1 intron-like protein |
UniProt Gene Name: | MT-CO1 |
UniProt Entry Name: | COX1_HUMAN |
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