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货号： bs-18295R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-18295R-RBITC
- 英文名称
- Anti-DLDD/Lipoamide Dehydrogenase/RBITC
- 中文名称
- 罗丹明（RBITC）标记的硫辛酰胺脱氢酶抗体
- 别 名
- Dehydrogenase complex, E3 component; Diaphorase; Dihydrolipoamide dehydrogenase; Dihydrolipoyl dehydrogenase; Dihydrolipoyl dehydrogenase mitochondrial; dld; DLDD; DLDH; DLDH_HUMAN; E3; E3 branched chain aplha-keto acid; E3 component of pyruvate dehydrogenase; E3 component of pyruvate dehydrogenase complex 2 oxo glutarate complex branched chain keto acid dehydrogenase complex; GCSL; Glycine cleavage system L protein; Glycine cleavage system protein L; LAD; lipoamide dehydrogenase; Lipoamide reductase; Lipoyl dehydrogenase; mitochondrial; OTTHUMP00000206744; OTTHUMP00000206746; OTTHUMP00000206748; OTTHUMP00000206749; PHE 3; PHE3; Pyruvate dehydrogenase component E3.
- 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 信号转导 新陈代谢 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Cow, Horse, Sheep, Xenopus laevis
- 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
- 50kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human DLDD/Lipoamide Dehydrogenase
- 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 member of the class-I pyridine nucleotide-disulfide oxidoreductase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. However, as a monomer, this protein can function as a protease. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]
Function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Subcellular Location:
Mitochondrion matrix.
Post-translational modifications:
Tyrosine phosphorylated.
DISEASE:
Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis.
Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:248600].
MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation.
Similarity:
Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family.
Database links:Entrez Gene: 1738Human
Entrez Gene: 403978Dog
Entrez Gene: 13382Mouse
Entrez Gene: 397129Pig
Entrez Gene: 298942Rat
Omim: 238331Human
SwissProt: P49819Dog
SwissProt: P09622Human
SwissProt: O08749Mouse
SwissProt: P09623Pig
SwissProt: Q6P6R2Rat
Unigene: 131711Human
Unigene: 3131Mouse
Unigene: 86962Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.