概述

  • 产品名称Anti-MTCO1抗体[11D8B7]
    参阅全部 MTCO1 一抗
  • 描述
    小鼠单克隆抗体[11D8B7] to MTCO1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Saccharomyces cerevisiae
    不与反应: Mouse, Rat, Human
  • 免疫原

    MTCO1 from S. cerevisiae denatured in SDS-PAGE

  • 阳性对照
    • Mitochondria from yeast membrane extract
  • 常规说明

    Product was previously marketed under the MitoSciences sub-brand.

性能

应用

Our Abpromise guarantee covers the use of ab110270 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 3 µg/ml. Predicted molecular weight: 57 kDa.

靶标

  • 功能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.
  • 通路Energy metabolism; oxidative phosphorylation.
  • 疾病相关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.
    Defects in MT-CO1 are a cause of anemia sideroblastic acquired idiopathic (AISA) [MIM:516030]; a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria.
    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].
  • 序列相似性Belongs to the heme-copper respiratory oxidase family.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt
  • 数据库链接
    • 别名
      • COI antibody
      • COX I antibody
      • COX1 antibody
      • COX1_HUMAN antibody
      • COXI antibody
      • Cytochrome c oxidase polypeptide I antibody
      • Cytochrome c oxidase subunit 1 antibody
      • Cytochrome C Oxidase subunit I antibody
      • Mitochondrially encoded cytochrome c oxidase I antibody
      • MT CO1 antibody
      • MT-CO1 antibody
      • MTCO 1 antibody
      • MTCO1 antibody
      see all

    Anti-MTCO1 antibody [11D8B7] 图像

    • Anti-MTCO1 antibody [11D8B7] (ab110270) at 3 µg/ml + Mitochondria from yeast membrane extract

      Predicted band size : 57 kDa

    Anti-MTCO1 antibody [11D8B7] (ab110270)参考文献

    This product has been referenced in:
    • Atkinson A  et al. Mzm1 influences a labile pool of mitochondrial zinc important for respiratory function. J Biol Chem 285:19450-9 (2010). Read more (PubMed: 20404342) »
    • Di Bella D  et al. Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28. Nat Genet 42:313-21 (2010). Read more (PubMed: 20208537) »

    See all 5 Publications for this product

    Product Wall

    I am sorry for the delay in getting back to you.

    I have now checked the other antibodies we have against this target and unfortunately none would be predicted to cross react with the Drosophila protein except the rabbit polyclonal ab90668. Ho...

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    Thank you for contacting us. These antibodies are indeed raised in mouse and these antibodies will work better in human, mouse, rat cells and tissue sections. In case you are planning to use these antibodies on mouse tissue sections then we wou...

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"