BCKDHB Antibody - N-terminal region
Product Sizes
25ul
£177.00
ARP58593-P050-25UL
100 ul
£412.00
ARP58593-P050-100UL
About this Product
- SKU:
- ARP58593-P050
- Additional Names:
- E1B; BCKDE1B; BCKDH E1-beta
- Clonality:
- Polyclonal
- Concentration:
- 0.5 mg/ml
- Extra Details:
- 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.
- Gene Details:
- branched chain keto acid dehydrogenase E1; beta polypeptide
- Host:
- Rabbit
- Immunogen:
- The immunogen is a synthetic peptide directed towards the N-terminal region of Human BCKDHB
- Molecular Weight:
- 23kDa
- Protein Details:
- 2-oxoisovalerate dehydrogenase subunit beta; mitochondrial Ensembl ENSP00000443564 Ensembl ENSP00000358775
- Purification:
- Affinity Purified
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- For short term use; store at 2-8C up to 1 week. For long term storage; store at -20C in small aliquots to prevent freeze-thaw cycles.
- Supplier:
- Aviva Systems Biology
- Type:
- Antibodies: Polyclonal Antibody
- Manufacturer's Data Sheet:html_datasheet.php
