BCKDHA purified MaxPab mouse polyclonal antibody (B01P)
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More Files
- More Functions
Western Blot (Transfected lysate)
Western Blot analysis of BCKDHA expression in transfected 293T cell line (H00000593-T01) by BCKDHA MaxPab polyclonal antibody.
Lane 1: BCKDHA transfected lysate(48.95 KDa).
Lane 2: Non-transfected lysate.
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Specification
Product Description
Mouse polyclonal antibody raised against a full-length human BCKDHA protein.
Immunogen
BCKDHA (NP_000700.1, 1 a.a. ~ 445 a.a) full-length human protein.
Sequence
MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYGNISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
Host
Mouse
Reactivity
Human
Quality Control Testing
Antibody reactive against mammalian transfected lysate.
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Transfected lysate)
Western Blot analysis of BCKDHA expression in transfected 293T cell line (H00000593-T01) by BCKDHA MaxPab polyclonal antibody.
Lane 1: BCKDHA transfected lysate(48.95 KDa).
Lane 2: Non-transfected lysate.
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Gene Info — BCKDHA
Entrez GeneID
593GeneBank Accession#
NM_000709.2Protein Accession#
NP_000700.1Gene Name
BCKDHA
Gene Alias
BCKDE1A, FLJ45695, MSU, MSUD1, OVD1A
Gene Description
branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Ontology
HyperlinkGene Summary
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene
Other Designations
2-oxoisovalerate dehydrogenase (lipoamide)|branched-chain alpha-keto acid dehydrogenase complex E1 alpha subunit|maple syrup urine disease
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Interactome
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Pathway
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Disease
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