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Rabbit Anti-MTND4L/FITC Conjugated antibody (bs-17890R-FITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-17890R-FITC
英文名稱 Rabbit Anti-MTND4L/FITC Conjugated antibody
中文名稱 FITC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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  神經生物學  信號轉導  激酶和磷酸酶  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, )
產品應用 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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