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  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 > 标记一抗 > 产品信息
Rabbit Anti-NUBPL/PE-Cy5 Conjugated antibody (bs-19505R-PE-Cy5)
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: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19505R-PE-Cy5
英文名称 Rabbit Anti-NUBPL/PE-Cy5 Conjugated antibody
中文名称 PE-Cy5标记的核苷酸结合蛋白样NUBPL抗体
别    名 C14orf127; FLJ12660; huInd1; IND1 homolog; Iron-sulfur protein NUBPL; Nubpl; NUBPL_HUMAN; nucleotide binding protein like; Nucleotide-binding protein-like.  
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  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Dog, Cow, Horse, Sheep, )
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 34kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NUBPL
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 member of the Mrp/NBP35 ATP-binding proteins family. The encoded protein is required for the assembly of the respiratory chain NADH dehydrogenase (complex I), an oligomeric enzymatic complex located in the inner mitochondrial membrane. Mutations in this gene cause mitochondrial complex I deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2014]

Function:
Required for the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). May deliver of one or more Fe-S clusters to complex I subunits.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Highest expression in liver and kidney. expressed at significant levels in small intestine and brain (at protein level).

DISEASE:
Defects in NUBPL 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 manifestations 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 Mrp/NBP35 ATP-binding proteins family.

Database links:

Entrez Gene: 80224 Human

Entrez Gene: 76826 Mouse

Entrez Gene: 299008 Rat

Omim: 613621 Human

SwissProt: Q8TB37 Human

SwissProt: Q9CWD8 Mouse

Unigene: 288981 Human

Unigene: 244781 Mouse

Unigene: 13455 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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    证书编号: 00124Q34771R2M/1100
通过国际医疗器械-质量管理体系ISO 13485:2016 GB/T 42061-2022    证书编号: CQC24QY10047R0M/1100
京ICP备05066980号-1         京公网安备110107000727号