NADH脱氢酶3抗体
规格：1mg/1ml
英文名： MT-ND3
别名： Mitochondrially encoded NADH dehydrogenase 3; MT-ND3; NADH dehydrogenase subunit 3; NADH-ubiquinone oxidoreductase chain 3; ND3; NU3M_HUMAN.
分子量： 13kDa
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 MT-ND3
交叉反应：Human,
细胞定位：
NADH脱氢酶3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 trans-location 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 3 (ND3) localizes to the hydrophobic protein fragment of complex I. Mutations in the gene encodiing for ND3 may be associated with Parkinson disease. Function: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysisNADH脱氢酶3抗体. 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-ND3 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in MT-ND3 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wideNADH脱氢酶3抗体 range of clinical disorders, from lethal neonatal disease to *****-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Similarity: Belongs to the complex I subunit 3 family. Gene ID: 4537 Database links: Entrez Gene: 4537 Human SwissProt: P03897 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产NADH脱氢酶3抗体品应用：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：细胞生物 信号转导 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid