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NUBPL Rabbit pAb (bs-19505R)  
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产品编号 bs-19505R
英文名称 NUBPL Rabbit pAb
中文名称 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.  
研究领域 细胞生物  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Human (predicted: Mouse,Rat,Sheep,Cow,Dog,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 34 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NUBPL: 1-100/319 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ 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 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.

SWISS:
Q8TB37

Gene ID:
80224

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



产品图片
Sample: Hela(Human) Cell Lysate at 30 ug Primary: Anti-NUBPL (bs-19505R) at 1/500 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 34 kD Observed band size: 34 kD
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