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Rabbit Anti-NDUFS1/Gold Conjugated antibody (bs-3890R-Gold)
订购热线:400-901-9800
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-3890R-Gold
英文名称 Anti-NDUFS1/Gold
中文名称 胶体金标记的线粒体复合物NDUFS1蛋白抗体
别    名 Complex I 75Kd; Complex I, mitochondrial respiratory chain, 75 kD subunit; NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) FeS protein 1 (75kD) (NADH coenzyme Q reductase) ; NADH ubiquinone oxidoreductase 75 kDa subunit mitochondrial; PRO1304.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 肿瘤  心血管  细胞生物  免疫学  神经生物学  结合蛋白  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Rat,  (predicted: Mouse, Dog, Pig, Rabbit, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 79kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Ndufs1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 background:
Ndufs1 belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. Ndufs1 has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in Ndufs1 gene are associated with complex I deficiency.

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). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized.

Subcellular Location:
Mitochondrion inner membrane.

Similarity:
Defects in NDUFS1 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.

Database links:

Entrez Gene: 4719 Human

Entrez Gene: 227197 Mouse

Entrez Gene: 301458 Rat

Omim: 157655 Human

SwissProt: P28331 Human

SwissProt: Q91VD9 Mouse

SwissProt: Q66HF1 Rat

Unigene: 471207 Human

Unigene: 598436 Human

Unigene: 290791 Mouse

Unigene: 1467 Rat



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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