BCKDHB Antibody - N-terminal region
Product Sizes
400ul
£597.00
OAAB00689-400UL
About this Product
- SKU:
- OAAB00689
- Additional Names:
- E1B; BCKDE1B; BCKDH E1-beta
- Clonality:
- Polyclonal
- Concentration:
- Varies by lot. See vial for concentration.
- 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.
- Host:
- Rabbit
- Immunogen:
- This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
- Isotype:
- Rabbit Ig
- Molecular Weight:
- 43 kDa
- Protein Details:
- 2-oxoisovalerate dehydrogenase subunit beta; mitochondrial
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
- Supplier:
- Aviva Systems Biology
- Type:
- Antibodies: Polyclonal Antibody
- Manufacturer's Data Sheet:html_datasheet.php
