Anti-NUBPL抗体-抗体-抗体-生物在线
Anti-NUBPL抗体

Anti-NUBPL抗体

商家询价

产品名称: Anti-NUBPL抗体

英文名称: NUBPL

产品编号: YB--19505R

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

上海钰博生物科技有限公司
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 Anti-NUBPL抗体

产品编号 YB-19505R
英文名称 NUBPL
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.  
规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
说 明 书 100ul  200ul
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, Mouse, Rat, Dog, Cow, Horse, Sheep, 
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 34kDa
细胞定位 细胞浆 
性    状 Lyophilized or 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
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.
PubMed PubMed
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 
 
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