DBT,支链α-酮酸脱氢酶E2抗体

产品编号HZ-14205R
英文名称DBT
中文名称支链α-酮酸脱氢酶E2抗体
别 名BCATE2; BCKAD E2 subunit; Branched chain acyltransferase, E2 component; Dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex; maple syrup urine disease); Dihydrolipoamide branched chain transacylase; Dihydrolipoamide branched chain transacylase E2; Dihydrolipoyl transacylase; Dihydrolipoyllysine residue (2 methylpropanoyl)transferase; E2; ODB2_HUMAN; E2 component of branched chain alpha keto acid dehydrogenase complex; E2B; Lipoamide acyltransferase component of mitochondrial branched chain alpha keto acid dehydrogenase complex; Mitochondrial branched chain alpha keto acid dehydrogenase transacylase subunit (E2b).
说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 信号转导 细胞类型标志物 新陈代谢 线粒体
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep,
DBT,支链α-酮酸脱氢酶E2抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量46kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human DBT
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.
DBT,支链α-酮酸脱氢酶E2抗体PubMedPubMed
产品介绍background:
The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008]

Function:
DBT or dihydrolipoamide branched chain transacylase E2 is a component of the BCKD (branched-chain alpha-keto dehydrogenase) complex which is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. The BCKD complex is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine.

Subcellular Location:
Mitochondrion matrix.

Gene ID:
1629

Database links:
Entrez Gene: 1629 Human
Entrez Gene: 280759 Cow
Entrez Gene: 13171 Mouse
Entrez Gene: 29611 Rat
Omim: 248610 Human
SwissProt: P11181 Cow
SwissProt: P11182 Human
SwissProt: P53395 Mouse
SwissProt: Q99PU6 Rat

DBT,支链α-酮酸脱氢酶E2抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.