验证数据展示
产品信息
68596-1-PBS targets BCKDHB as part of a matched antibody pair:
MP50570-1: 68596-2-PBS capture and 68596-1-PBS detection (validated in Cytometric bead array)
MP50570-3: 68596-4-PBS capture and 68596-1-PBS detection (validated in Cytometric bead array)
Unconjugated mouse monoclonal antibody pair in PBS only (BSA and azide free) storage buffer at a concentration of 1 mg/mL, ready for conjugation.
This conjugation ready format makes antibodies ideal for use in many applications including: ELISAs, multiplex assays requiring matched pairs, mass cytometry, and multiplex imaging applications.Antibody use should be optimized by the end user for each application and assay.
| 经测试应用 | Cytometric bead array, Indirect ELISA Application Description |
| 经测试反应性 | human, mouse, rat |
| 免疫原 |
CatNo: Ag33802 Product name: Recombinant human BCKDHB protein Source: e coli.-derived, PET28a Tag: 6*His Domain: 1-392 aa of BC040139 Sequence: 种属同源性预测 |
| 宿主/亚型 | Mouse / IgG1 |
| 抗体类别 | Monoclonal |
| 产品类型 | Antibody |
| 全称 | branched chain keto acid dehydrogenase E1, beta polypeptide |
| 别名 | E1B, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDH E1-beta, BCKDE1B, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial |
| 计算分子量 | 392 aa, 43 kDa |
| 观测分子量 | 35-37 kDa |
| GenBank蛋白编号 | BC040139 |
| 基因名称 | BCKDHB |
| Gene ID (NCBI) | 594 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein G purification |
| UNIPROT ID | P21953 |
| 储存缓冲液 | PBS only, pH 7.3. |
| 储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
Branched-chain alpha-keto acid dehydrogenase E1 component beta chain (BCKDHB; BCKDE1B; BCKDH E1-beta) is a subunit of the BCKDH complex, which is a mitochondrial enzyme in the degradation pathway for branched-chain amino acids (BCAA). Together with BCKDHA, BCKDHB forms the E1 subunit of this complex, whereas DBT and DLD are the E2 and E3 subunits, respectively. A deficiency of the BCKDH complex in humans causes maple syrup urine disease (MSUD), a severe neurometabolic disorder diagnosed by the detection alloisoleucine in plasma (MIM 248600). (PMID: 30709776)
