Recombinant人COX15 protein (ab116958)

概述

  • 产品名称Recombinant人COX15 protein
  • 蛋白长度Protein fragment

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionQ7KZN9
    • 种属Human
    • 序列LTESGLSMVDWHLIKEMKPPTSQEEWEAEFQRYQQFPEFKILNHDMTLTE FKFIWYMEYSH
    • 分子量32 kDa including tags
    • 氨基酸92 to 152

技术指标

Our Abpromise guarantee covers the use of ab116958 in the following tested applications.

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

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • CEMCOX2
    • COX15
    • COX15 homolog, cytochrome c oxidase assembly protein
    • COX15, S. cerevisiae, homolog of
    • COX15_HUMAN
    • cytochrome c oxidase assembly homolog 15 (yeast)
    • Cytochrome c oxidase assembly protein COX15 homolog
    • cytochrome c oxidase subunit 15
    see all
  • 功能May be involved in the biosynthesis of heme A.
  • 组织特异性Predominantly found in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.
  • 通路Porphyrin metabolism; heme A biosynthesis; heme A from heme O: step 1/1.
  • 疾病相关Defects in COX15 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 COX15 are a cause of Leigh syndrome (LS) [MIM:256000]. An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.
  • 序列相似性Belongs to the COX15/CtaA family.
  • 细胞定位Mitochondrion membrane.
  • Information by UniProt

Recombinant Human COX15 protein 图像

  • 12.5% SDS-PAGE showing ab116958 at approximately 32.34kDa stained with Coomassie Blue.

Recombinant Human COX15 protein (ab116958)参考文献

ab116958 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab116958.
Please use the links above to contact us or submit feedback about this product.

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