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    Rabbit Anti-DAP13/BF555 Conjugated antibody (bs-14196R-BF555)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-14196R-BF555
    英文名稱(chēng) Rabbit Anti-DAP13/BF555 Conjugated antibody
    中文名稱(chēng) BF555標記的細胞分化相關(guān)蛋白13抗體
    別    名 13 kDa differentiation associated protein; 13 kDa differentiation-associated protein; 2410011G03Rik; AW112974; B17.2; CI-B17.2; CIB17.2; Complex I B17.2; Complex I-B17.2; DAP13; MGC107642; MGC7999; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12; NADH ubiquinone oxidoreductase subunit B17.2; NADH-ubiquinone oxidoreductase subunit B17.2; NDUAC_HUMAN; NDUFA12; RGD1311462.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 腫瘤  細胞生物  神經(jīng)生物學(xué)  信號轉導  新陳代謝  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Rat, )
    產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 17kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human DAP13
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
    保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    產(chǎn)品介紹 background:
    This gene encodes a protein which is part of mitochondrial complex 1, part of the oxidative phosphorylation system in mitochondria. Complex 1 transfers electrons to ubiquinone from NADH which establishes a proton gradient for the generation of ATP. Mutations in this gene are associated with Leigh syndrome due to mitochondrial complex 1 deficiency. Pseudogenes of this gene are located on chromosomes 5 and 13. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2012]

    Function:
    Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.

    Subcellular Location:
    Mitochondrion inner membrane.

    DISEASE:
    Defects in NDUFA12 are the 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.

    Similarity:
    Belongs to the complex I NDUFA12 subunit family.

    Database links:

    Entrez Gene: 55967 Human

    Entrez Gene: 281742 Cow

    Entrez Gene: 66414 Mouse

    Entrez Gene: 299739 Rat

    Omim: 614530 Human

    SwissProt: O97725 Cow

    SwissProt: Q9UI09 Human

    SwissProt: Q7TMF3 Mouse

    Unigene: 506374 Human

    Unigene: 27886 Mouse

    Unigene: 461501 Mouse



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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