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Rabbit Anti-NUBPL/BF350 Conjugated antibody (bs-19505R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-19505R-BF350
英文名稱 Rabbit Anti-NUBPL/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的核苷酸結(jié)合蛋白樣NUBPL抗體
別    名 C14orf127; FLJ12660; huInd1; IND1 homolog; Iron-sulfur protein NUBPL; Nubpl; NUBPL_HUMAN; nucleotide binding protein like; Nucleotide-binding protein-like.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,  (predicted: Mouse, Rat, Dog, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NUBPL
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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.
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