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|Tested species reactivity||Human|
|Host / Isotype||Rabbit / IgG|
|Immunogen||KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB|
|Purification||Antigen affinity chromatography|
|Contains||0.09% sodium azide|
|Storage Conditions||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C|
|Tested Applications||Dilution *|
|Flow Cytometry (Flow)||1:10-1:50|
|Western Blot (WB)||1:1000|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
2-oxoisovalerate dehydrogenase beta subunit; 2-oxoisovalerate dehydrogenase subunit beta; BCKDE1B; BCKDH E1-beta; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched chain keto acid dehydrogenase E1, beta polypeptide; branched-chain alpha-ke; branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1; E1B; E1b-beta subunit of the branched-chain complex; mitochondrial; testis secretory sperm-binding protein Li 240mP
BCKDE1B; BCKDH E1-beta; BCKDHB; E1B