ND4L抗体
规格：1mg/1ml
英文名： MTND4L
别名： EC=1.6.5.3; MT-ND4L; NADH dehydrogenase subunit 4L; NADH-ubiquinone oxidoreductase chain 4L; NADH4L; ND4L; NU4LM_HUMAN.
分子量： 11kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MTND4L
交叉反应：Human,
细胞定位：细胞膜
ND4L抗体产品介绍：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, ND4L抗体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 ND4L抗体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. Gene ID: 4539 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.
ND4L抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 细胞生物 神经生物学 信号转导 激酶和磷酸酶
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid