BCKDHA Antibody - N-terminal region (ARP56119_P050)

• Product Number: ARP56119_P050
• Name: BCKDHA Antibody - N-terminal region (ARP56119_P050)
• Protein Size (# AA): 445 amino acids
• Molecular Weight: 50kDa
• Subunit: alpha, mitochondrial
• NCBI Gene Id: 593
• Host: Rabbit
• Clonality: Polyclonal
• Concentration: 0.5 mg/ml
• Gene Full Name: Branched chain keto acid dehydrogenase E1, alpha polypeptide
• Alias Symbols: MSU, MSUD1, OVD1A, BCKDE1A
• Peptide Sequence: Synthetic peptide located within the following region: NVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILY
• Product Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Reference: Flaschker,N., (2007) J. Inherit. Metab. Dis. 30 (6), 903-909
• Description of Target: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998 [PubMed 9582350]). The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.[supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications. PRIMARYREFSEQ_SPAN PRIMARY_IDENTIFIER PRIMARY_SPAN COMP 1-675 BI910860.1 12-686 676-1196 BG742673.1 80-600 1197-1731 BM702667.1 48-582 1732-1763 BE223026.1 1-32 c 1764-1781 BQ018849.1 1-18 c
• Protein Interactions: UBC; CUL3; BCKDHB; BCKDK;
• Reconstitution and Storage: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
• Blocking Peptide: For anti-BCKDHA (ARP56119_P050) antibody is Catalog # AAP56119 (Previous Catalog # AAPP37740)
• Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human BCKDHA
• Uniprot ID: P12694
• Protein Name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
• Sample Type Confirmation:

  BCKDHA is supported by BioGPS gene expression data to be expressed in MCF7

• Protein Accession #: NP_000700
• Purification: Affinity Purified
• Nucleotide Accession #: NM_000709
• Tested Species Reactivity: Human
• Gene Symbol: BCKDHA
• Predicted Species Reactivity: Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Sheep, Zebrafish
• Application: IHC, WB
• Predicted Homology Based on Immunogen Sequence: Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Sheep: 100%; Zebrafish: 93%
• Image 1: MCF7, HepG2
  
  Host: Rabbit
  Target: BCKDHA
  Positive control (+): MCF7 (N10)
  Negative control (-): HepG2 (HG)
  Antibody concentration: 1ug/ml
• Image 2: Human MCF-7
  
  WB Suggested Anti-BCKDHA Antibody Titration: 0.2-1 ug/ml
  ELISA Titer: 1:312500
  Positive Control: MCF7 cell lysateBCKDHA is supported by BioGPS gene expression data to be expressed in MCF7
• Image 3: Human Adult Placenta
  
  Host: Rabbit
  Target Name: SERPINA3
  Sample Type: Human Adult Placenta
  Antibody Dilution: 1.0ug/ml
• Image 4: Human Jurkat Whole Cell
  
  Host: Rabbit
  Target Name: BCKDHA
  Sample Tissue: Human Jurkat Whole Cell
  Antibody Dilution: 1ug/ml
• Image 5: Human Lung Tissue
  
  Rabbit Anti-BCKDHA Antibody
  Catalog Number: ARP56119_P050
  Formalin Fixed Paraffin Embedded Tissue: Human Lung Tissue
  Observed Staining: Cytoplasmic in alveolar type I & II cells
  Primary Antibody Concentration: 1:100
  Secondary Antibody: Donkey anti-Rabbit-Cy3
  Secondary Antibody Concentration: 1:200
  Magnification: 20X
  Exposure Time: 0.5 - 2.0 sec