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Rabbit Anti-Mimitin/APC Conjugated antibody (bs-6551R-APC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-6551R-APC
英文名称1 Rabbit Anti-Mimitin/APC Conjugated antibody
中文名称 APC标记的MYC诱导线粒体蛋白抗体
别    名 mitochondrial; B17.2 like; B17.2-like; B17.2L; MIMIT_HUMAN; Mimitin; Mimitin mitochondrial; MMTN; Myc induced mitochondrial protein; Myc-induced mitochondrial protein; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex assembly factor 2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; NDUFA12 like; NDUFA12 like protein; NDUFA12-like protein; NDUFA12L; NDUFAF2.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  信号转导  肿瘤细胞生物标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, )
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 20kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Mimitin (75-123aa)
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:
NADH:ubiquinone oxidoreductase (complex I) catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q) in the first step of the mitochondrial respiratory chain, resulting in the translocation of protons across the inner mitochondrial membrane. This gene encodes a complex I assembly factor. Mutations in this gene cause progressive encephalopathy resulting from mitochondrial complex I deficiency.

Function:
Acts as a molecular chaperone for mitochondrial complex I assembly.

Subunit:
Mitochondrion.

Subcellular Location:
Highly expressed in ESCC cells. Also expressed in heart, skeletal muscle, liver, and in fibroblasts.

DISEASE:
Defects in NDUFAF2 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 disorders, 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 complex I NDUFA12 subunit family.

Database links:
UniProtKB/Swiss-Prot: Q8N183.1

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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