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    Rabbit Anti-MTND4L/Cy5 Conjugated antibody (bs-17890R-Cy5)
    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-17890R-Cy5
    英文名稱(chēng) Rabbit Anti-MTND4L/Cy5 Conjugated antibody
    中文名稱(chēng) Cy5標記的ND4L抗體
    別    名 EC=1.6.5.3; MT-ND4L; NADH dehydrogenase subunit 4L; NADH-ubiquinone oxidoreductase chain 4L; NADH4L; ND4L; NU4LM_HUMAN.  
    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 心血管  細胞生物  神經(jīng)生物學(xué)  信號轉導  激酶和磷酸酶  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, )
    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 11kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human MTND4L
    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:
    NADH:ubiquinone oxidoreductase (complex I) is an extremely complicated multiprotein complex located in the inner mitochondrial membrane. Human complex I is important for energy metabolism because its main function is to transport electrons from NADH to ubiquinone, which is accompanied by translocation of protons from the mitochondrial matrix to the intermembrane space. Human complex I appears to consist of 41 subunits. A small number of complex I subunits are the products of mitochondrial genes (subunits 1-7), while the remainder are nuclear encoded and imported from the cytoplasm. NADH dehydrogenase subunit 4L (ND4L) is most likely a component of the hydrophobic protein fragment of Complex I. Mutations in the gene encodiing for ND4 are implicated in Leber hereditary optic neuropathy, a rare condition that can cause loss of central vision.

    Function:
    Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 membrane.

    DISEASE:
    Defects in MT-ND4L 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.

    Similarity:
    Belongs to the complex I subunit 4L family.

    Database links:

    Entrez Gene: 4539 Human

    Omim: 516004 Human

    SwissProt: P03901 Human



    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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