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货号： bs-19077R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19077R-RBITC
- 英文名称
- Anti-NDUFAF6/RBITC
- 中文名称
- 罗丹明（RBITC）标记的NDUFAF6蛋白抗体
- 别 名
- CCDC113; C8orf38; NDUF6_HUMAN; Coiled-coil domain-containing protein 113 Gene names; DKFZp434N1418; HSPC065; NADH dehydrogenase (ubiquinone) complex I assembly factor 6; Putative phytoene synthase; UPF0551 protein C8orf38 mitochondrial.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 神经生物学 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- 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
- 33kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human NDUFAF6
- 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:
This gene encodes a protein that localizes to mitochondria and contains a predicted phytoene synthase domain. The encoded protein plays an important role in the assembly of complex I (NADH-ubiquinone oxidoreductase) of the mitochondrial respiratory chain through regulation of subunit ND1 biogenesis. Mutations in this gene are associated with complex I enzymatic deficiency. [provided by RefSeq, Nov 2011]
Function:
Involved in the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I) at early stages. May play a role in the biogenesis of MT-ND1.
Subcellular Location:
Isoform 1: Mitochondrion inner membrane. Note: Peripherally localized on the matrix face of the mitochondrial inner membrane. Ref.7
Isoform 2: Cytoplasm. Nucleus
DISEASE:
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.Note: The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the NDUFAF6 family.
Database links:Entrez Gene: 137682Human
Entrez Gene: 523017Cow
Entrez Gene: 76947Mouse
Entrez Gene: 297821Rat
Omim: 612392Human
SwissProt: A7YVD7Cow
SwissProt: Q330K2Human
SwissProt: A2AIL4Mouse
SwissProt: D3ZN43Rat
Unigene: 729144Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.