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Anti-BCKDHA antibody produced in mouse
| 产品编号： | 3347541 |
| 规格： | purified immunoglobulin, buffered aqueous solution |
| 包装规格： | 50 μG |
| 产品类别： | 进口试剂 |
| 品牌： | Sigma-Aldrich |
| 优惠价： | 立即咨询 |
产品别名
Anti-BCKDHA antibody produced in mouse
BCKDE1A
OVD1A
FLJ45695
MSUD1
MSU
基本信息
| NACRES | NA.41 |
| General description【一般描述】 | The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene |
| Immunogen【免疫原】 | BCKDHA (NP_000700.1, 1 a.a. ~ 445 a.a) full-length human protein. Sequence MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYGNISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK |
| Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
产品性质
| biological source【生物来源】 | mouse |
| conjugate【偶联物】 | unconjugated |
| antibody form【抗体形式】 | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone【克隆】 | polyclonal |
| form【形式】 | buffered aqueous solution |
| mol wt【分子量】 | antigen ~50.5 kDa |
| species reactivity | human |
| technique(s) | western blot: 1 μg/mL |
| NCBI accession no.【NCBI登记号】 | NM_000709.2 |
| UniProt accession no.【UniProt登记号】 | P12694 |
| shipped in【运输】 | dry ice |
| storage temp.【储存温度】 | −20℃ |
| Gene Information | human ... BCKDHA(593) |
安全信息
| Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
