Rabbit BCKDHB Polyclonal Antibody | anti-BCKDHB antibody
BCKDHB Antibody - N-terminal region
Gene Names
BCKDHB; E1B; BCKDE1B; BCKDH E1-beta
Reactivity
Cow, Dog, Horse, Human, Pig, Rabbit, Rat
Applications
Western Blot
Purity
Affinity Purified
Synonyms
BCKDHB; Polyclonal Antibody; BCKDHB Antibody - N-terminal region; anti-BCKDHB antibody
Host
Rabbit
Reactivity
Cow, Dog, Horse, Human, Pig, Rabbit, Rat
Clonality
Polyclonal
Purity/Purification
Affinity Purified
Form/Format
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Sequence
Synthetic peptide located within the following region: GFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDN
Sequence Length
218
Applicable Applications for anti-BCKDHB antibody
Western Blot (WB)
Homology
Cow: 93%; Dog: 93%; Horse: 93%; Human: 100%; Pig: 86%; Rabbit: 93%; Rat: 79%
Immunogen
The immunogen is a synthetic peptide directed towards the N-terminal region of Human BCKDHB
Preparation and Storage
For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.
Western Blot (WB)
(Host: RabbitTarget Name: BCKDHBSample Type: Thyroid tumor lysatesAntibody Dilution: 1.0ug/ml)
Related Product Information for anti-BCKDHB antibody
This is a rabbit polyclonal antibody against BCKDHB. It was validated on Western Blot
Target 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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.
Target 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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.
Product Categories/Family for anti-BCKDHB antibody
NCBI and Uniprot Product Information
NCBI GI #
NCBI GeneID
Molecular Weight
23kDa
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1 subunit beta
NCBI Official Symbol
BCKDHB
NCBI Official Synonym Symbols
E1B; BCKDE1B; BCKDH E1-beta
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
NCBI Description
This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016]
