|Application ||WB, IHC-P, FC, IF, E|
|Calculated MW||43122 Da|
|Antigen Region||41-70 aa|
|Other Names||2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB|
|Target/Specificity||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.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||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.|
|Precautions||BCKDHB Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
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.
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :
Gorzelany, K., et al. Turk. J. Pediatr. 51(2):97-102(2009)
Quental, S., et al. Mol. Genet. Metab. 94(2):148-156(2008)
Kang, H., et al. Fertil. Steril. 89(3):728-731(2008)
Flaschker, N., et al. J. Inherit. Metab. Dis. 30(6):903-909(2007)
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