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细胞色素c氧化酶1抗体Ys-3953R
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产品编号Ys-3953R
英文名称COX1/MTCO1
中文名称细胞色素c氧化酶1抗体
别 名COI; COX I; COXI; Cytochrome oxidase 1; Cytochrome c oxidase polypeptide I; Cytochrome C Oxidase subunit I; Mitochondrially encoded cytochrome c oxidase I; MT CO1; MTCO 1; MTCO1; MT-CO1; MTCO1; Cytochrome c oxidase subunit 1; COX1_HUMAN.
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse, Rat, (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量57kDa
细胞定位细胞浆 细胞膜 线粒体
性 状Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human COX1: 401-500/513
亚 型IgG
纯化方法affinity purified by Protein A
缓 冲 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMedPubMed
产品介绍Cytochrome c oxidase subunit I (COI or MTCO1) is one of three mitochondrial DNA (mtDNA) encoded subunits (MTCO1, MTCO2, MTCO3) of respiratory Complex IV. Complex IV is located within the mitochondrial inner membrane and is the third and final enzyme of the electron transport chain of mitochondrial oxidative phosphorylation. Complex IV is composed of 13 polypeptides. Subunits I, II, and III (MTCO1, MTCO2, MTCO3) are encoded by mtDNA while subunits IV, Va, Vb, VIa, VIb, VIc, VIIa, VIIb, VIIc, and VIII are nuclear encoded. Mammalian MTCO1 has 12 membrane-spanning alpha-helices (I to XII).
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. 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 biｍmbedllic center ｆormed by heme A3 and copper B.
Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.
DISEASE:
Defects in MT-CO1 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. 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.
Note=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) [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 Leigh syndrome.
Defects in MT-CO1 are associated with recurrent myoglobinuria mitochondrial (RM-MT) [MIM:550500]. 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) [MIM:500008]. 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) [MIM:114500].
Similarity:
Belongs to the heme-copper respiratory oxidase family.
SWISS:
P00395
Gene ID:
4512
热门标签：细胞色素c氧化酶1抗体,抗体
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