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Rabbit Anti-NDUF3/PE Conjugated antibody (bs-19067R-PE)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19067R-PE
英文名称 Anti-NDUF3/PE
中文名称 PE标记的NDUF3蛋白抗体
别    名 C3orf60; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3; NDUF3_HUMAN; ndufaf3.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  转录调节因子  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Pig, Horse, Rabbit, )
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 20kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NDUF3
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.
产品介绍 background:
This gene encodes a mitochondrial complex I assembly protein that interacts with complex I subunits. Mutations in this gene cause mitochondrial complex I deficiency, a fatal neonatal disorder of the oxidative phosphorylation system. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2009]

Function:
Essential factor for the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I).

Subunit:
Interacts with NDUFAF4, NDUFS2 and NDUFS3.

Subcellular Location:
Nucleus. Mitochondrion inner membrane.

DISEASE:
Defects in NDUFAF3 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: 25915 Human

Entrez Gene: 56769 Rat

Omim: 612911 Human

SwissProt: Q9BU61 Human

SwissProt: O08776 Rat

Unigene: 31387 Human

Unigene: 40118 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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