Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations [0]
Submit
Validation data
Reference
Comment
Report error
- Product number
- abx025837 - Provider product page
- Provider
- Abbexa
- Product name
- 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
- Antibody type
- Polyclonal
- 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.
- Reactivity
- Human
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 80 µl
- Storage
- Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
No comments: Submit comment
No validations: Submit validation data