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Rabbit Anti-DAP13/PE Conjugated antibody (bs-14196R-PE)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-14196R-PE
英文名稱 Rabbit Anti-DAP13/PE Conjugated antibody
中文名稱 PE標記的細胞分化相關蛋白13抗體
別    名 13 kDa differentiation associated protein; 13 kDa differentiation-associated protein; 2410011G03Rik; AW112974; B17.2; CI-B17.2; CIB17.2; Complex I B17.2; Complex I-B17.2; DAP13; MGC107642; MGC7999; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12; NADH ubiquinone oxidoreductase subunit B17.2; NADH-ubiquinone oxidoreductase subunit B17.2; NDUAC_HUMAN; NDUFA12; RGD1311462.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  神經生物學  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Rat, )
產品應用 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 17kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DAP13
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.
產品介紹 background:
This gene encodes a protein which is part of mitochondrial complex 1, part of the oxidative phosphorylation system in mitochondria. Complex 1 transfers electrons to ubiquinone from NADH which establishes a proton gradient for the generation of ATP. Mutations in this gene are associated with Leigh syndrome due to mitochondrial complex 1 deficiency. Pseudogenes of this gene are located on chromosomes 5 and 13. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2012]

Function:
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.

Subcellular Location:
Mitochondrion inner membrane.

DISEASE:
Defects in NDUFA12 are the cause of Leigh syndrome (LS) [MIM:256000]. An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.

Similarity:
Belongs to the complex I NDUFA12 subunit family.

Database links:

Entrez Gene: 55967 Human

Entrez Gene: 281742 Cow

Entrez Gene: 66414 Mouse

Entrez Gene: 299739 Rat

Omim: 614530 Human

SwissProt: O97725 Cow

SwissProt: Q9UI09 Human

SwissProt: Q7TMF3 Mouse

Unigene: 506374 Human

Unigene: 27886 Mouse

Unigene: 461501 Mouse



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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