产品货号 :  mlR19093

英文名称 :  NDUFS8

中文名称 :  NDUFS8蛋白抗体

别    名 :  CI 23kD; CI-23kD; Complex I 23kD; Complex I-23kD; EC 1.6.5.3; EC 1.6.99.3; Human mitochondrial NADH dehydrogenase ubiquinone Fe S protein 8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial; NADH ubiquinone oxidoreductase 23 kDa subunit; NADH-ubiquinone oxidoreductase 23 kDa subunit; NDUFS8; NDUS8_HUMAN; TYKY; TYKY subunit; Ubiquinoneiron sulfur protein 8, mitochondrial precursor.  

研究领域 :  肿瘤  细胞生物  信号转导  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :  Human,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.

分 子 量 :  12kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human NDUFS8:1-100/210

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  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.

PubMed :  PubMed

产品介绍 :  This gene encodes a subunit of mitochondrial NADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzyme of the respiratory chain responsible for NADH oxidation, ubiquinone reduction, and the ejection of protons from mitochondria. The encoded protein is involved in the binding of two of the six to eight iron-sulfur clusters of Complex I and, as such, is required in the electron transfer process. Mutations in this gene have been associated with Leigh syndrome. [provided by RefSeq, Mar 2010]

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 (By similarity). May donate electrons to ubiquinone.

Subcellular Location:

Mitochondrion.

DISEASE:

Defects in NDUFS8 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.

Similarity:

Belongs to the complex I 23 kDa subunit family.

Contains 2 4Fe-4S ferredoxin-type domains.

SWISS:

O00217

Gene ID:

4728

Important Note:

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