扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  人才招聘  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 一抗 > 产品信息
Rabbit Anti-NDUF3  antibody (bs-19067R)
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
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-19067R
英文名称 NDUF3
中文名称 NDUF3蛋白抗体
别    名 C3orf60; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3; NDUF3_HUMAN; ndufaf3.  
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 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 20kDa
细胞定位 细胞核 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NDUF3: 51-150/184 
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 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.

SWISS:
Q9BU61

Gene ID:
25915

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



版权所有 2004-2026 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    认证编号: 00122Q31509R1M/1100
京ICP备05066980号-1         京公网安备110107000727号