BCKDHB Antibody
Product Specifications
Background
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.
NCBI Gene ID
594
Swiss Prot
P21953
Accession Number
P21953
Host
Rabbit
Reactivity
Human
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.
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Obesity, Signal Transduction
Purification
This antibody is purified through a protein A column, followed by peptide affinity purification.
Concentration
Batch dependent
Buffer
Supplied in PBS with 0.09% (W/V) sodium azide.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Product Datasheet
https://www.prosci-inc.com/?datasheet_sku=55-327
Calculated Molecular Weight
43 kDa
Fragment
Rabbit Ig
Symbol
BCKDHB
NCBI Official Name
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
NCBI Organism
Homo sapiens
Background Reference 01
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :
Background Reference 02
Gorzelany, K., et al. Turk. J. Pediatr. 51 (2) :97-102 (2009)
Background Reference 03
Quental, S., et al. Mol. Genet. Metab. 94 (2) :148-156 (2008)
Background Reference 04
Kang, H., et al. Fertil. Steril. 89 (3) :728-731 (2008)
Other Product Names
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB
Tested Applications
WB, IHC-P, IF, Flow
Protein ID
129034
Physical Properties
Liquid
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