Recombinant Human BCKDHB Protein, GST-tagged
Cat.No. : | BCKDHB-137H |
Product Overview : | Human BCKDHB full-length ORF ( AAH40139, 1 a.a. - 392 a.a.) recombinant protein with GST-tag at N-terminal. |
- Specification
- Gene Information
- Related Products
Description : | Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3 non-coding regions, but encoding the same isoform. |
Source : | Wheat Germ |
Species : | Human |
Tag : | GST |
Form : | 50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer. |
Molecular Mass : | 68.86 kDa |
AA Sequence : | MAVVAAAAGWLLRLRAAGAEGHWRR LPGAGLARGFLHPAATVEDAAQRRQ VAHFTFQPDPEPREYGQTQKMNLFQ SVTSALDNSLAKDPTAVIFGEDVAF GGVFRCTVGLRDKYGKDRVFNTPLC EQGIVGFGIGIAVTGATAIAEIQFA DYIFPAFDQIVNEAAKYRYRSGDLF NCGSLTIRSPWGCVGHGALYHSQSP EAFFAHCPGIKVVIPRSPFQAKRLL LSCIEDKNPCIFFEPKILYRAAAEE VPIEPYNIPLSQAEVIQEGSDVTLV AWGTQVHVIREVASMAKEKLGVSCE VIDLRTIIPWDVDTICKSVIKTGRL LISHEAPLTGGFASEISSTVQEECF LNLEAPISRVCGYDTPFPHIFEPFY IPDKWKCYDALRKMINY |
Notes : | Best use within three months from the date of receipt of this protein. |
Storage : | Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing. |
Storage Buffer : | 50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer. |
Gene Name : | BCKDHB branched chain keto acid dehydrogenase E1, beta polypeptide [ Homo sapiens ] |
Official Symbol : | BCKDHB |
Synonyms : | BCKDHB; branched chain keto acid dehydrogenase E1, beta polypeptide; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; maple syrup urine disease; BCKDE1B; BCKDH E1-beta; 2-oxoisovalerate dehydrogenase beta subunit; E1b-beta subunit of the branched-chain complex; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched-chain alpha-keto acid dehydrogenase E1 component beta chain; E1B; dJ279A18.1; FLJ17880; |
Gene ID : | 594 |
mRNA Refseq : | NM_000056 |
Protein Refseq : | NP_000047 |
MIM : | 248611 |
UniProt ID : | P21953 |
Products Types
◆ Recombinant Protein | ||
BCKDHB-436H | Recombinant Human BCKDHB Protein, His (Fc)-Avi-tagged | +Inquiry |
BCKDHB-612R | Recombinant Rat BCKDHB Protein, His (Fc)-Avi-tagged | +Inquiry |
BCKDHB-985M | Recombinant Mouse BCKDHB Protein, His (Fc)-Avi-tagged | +Inquiry |
BCKDHB-6225C | Recombinant Chicken BCKDHB | +Inquiry |
BCKDHB-2332M | Recombinant Mouse BCKDHB Protein | +Inquiry |
◆ Lysates | ||
BCKDHB-8493HCL | Recombinant Human BCKDHB 293 Cell Lysate | +Inquiry |
BCKDHB-8492HCL | Recombinant Human BCKDHB 293 Cell Lysate | +Inquiry |
Related Gene
For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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