BCKDHA (Human) Recombinant Protein (P01)
产品名称： BCKDHA (Human) Recombinant Protein (P01)
英文名称： BCKDHA (Human) Recombinant Protein (P01)
产品编号： H00000593-P01
产品价格： 0
产品产地： 台湾
品牌商标： Abnova
更新时间： null
使用范围： null
亚诺法生技股份有限公司（Abnova)
- 联系人 :
- 地址 : 台湾台北市内湖区洲子街 108 号 9 楼
- 邮编 : 11493
- 所在区域 : 台湾
- 电话 : +886-920**1152 点击查看
- 传真 : 点击查看
- 邮箱 : sales@abnova.com.tw
- Specification
- Product Description:
- Human BCKDHA full-length ORF ( AAH07878.1, 1 a.a. - 445 a.a.) recombinant protein with GST-tag at N-terminal.
- Sequence:
- MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYGNISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
- Theoretical MW (kDa):
- 74.69
- Preparation Method:
- in vitro wheat germ expression system
- Purification:
- Glutathione Sepharose 4 Fast Flow
- Storage Buffer:
- 50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.
- Storage Instruction:
- Store at -80°C. Aliquot to avoid repeated freezing and thawing.
- Quality Control Testing:
- 12.5% SDS-PAGE Stained with Coomassie Blue.
- Note:
- Best use within three months from the date of receipt of this protein.
- MSDS:
Download
- Application Image
- Enzyme-linked Immunoabsorbent Assay
- Western Blot (Recombinant protein)
- Antibody Production
- Protein Array
- Entrez GeneID:
- 593
- GeneBank Accession#:
- BC007878
- Protein Accession#:
- AAH07878.1
- Gene Name:
- BCKDHA
- Gene Alias:
- BCKDE1A,FLJ45695,MSU,MSUD1,OVD1A
- Gene Description:
- branched chain keto acid dehydrogenase E1, alpha polypeptide
- Gene Ontology:
- Hyperlink
- Gene Summary:
- 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
- Other Designations:
- 2-oxoisovalerate dehydrogenase (lipoamide),branched-chain alpha-keto acid dehydrogenase complex E1 alpha subunit,maple syrup urine disease
- Related Disease