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.

Target	2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB)
Clonality	Polyclonal
Reactivity	Human
Tested Applications	ELISA, WB, IHC, IF/ICC, FCM
Host	Rabbit
Recommended dilutions	WB: 1/1000, IHC-P: 1/500, IF/ICC: 1/25, FCM: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user.
Conjugation	Unconjugated
Immunogen	KLH-conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
Isotype	IgG
Form	Liquid
Purification	Purified through a protein A column, followed by peptide affinity purification.
Storage	Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
UniProt Primary AC	P21953 (UniProt, ExPASy)
UniProt Secondary AC	Q5T2J3, Q9BQL0
UniProt Entry Name	ODBB_HUMAN
Gene Symbol	BCKDHB
KEGG	hsa:594
String	9606.ENSP00000318351
Molecular Weight	Calculated MW: 43.1 kDa
Buffer	PBS containing 0.09% sodium azide.
Availability	Shipped within 5-10 working days.
Note	This product is for research use only.

Research Articles on 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB)