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Rabbit Anti-MT-ND3  antibody (bs-17867R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-17867R
英文名称 MT-ND3
中文名称 NADH脱氢酶3抗体
别    名 Mitochondrially encoded NADH dehydrogenase 3; MT-ND3; NADH dehydrogenase subunit 3; NADH-ubiquinone oxidoreductase chain 3; ND3; NU3M_HUMAN.  
研究领域 细胞生物  信号转导  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human)
产品应用 IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 13kDa
细胞定位 细胞浆 线粒体
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MT-ND3: 1-80/115 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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 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-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 wide range of clinical disorders, from lethal neonatal disease to adult-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.

SWISS:
P03897

Gene ID:
4537

Database links:

Entrez Gene: 4537 Human

Entrez Gene: 17718 Mouse

Entrez Gene: 26199 Rat

SwissProt: P03897 Human

SwissProt: P03899 Mouse

SwissProt: P05506 Rat




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