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BCKDHB Antibody (N-term)

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • 59 - BCKDHB Antibody (N-term) AP10672a
    Immunohistochemical analysis of paraffin-embedded Human hepatocarcinoma tissue using AP10672a performed on the Leica® BOND RXm. Tissue was fixed with formaldehyde at room temperature, antigen retrieval was by heat mediation with a EDTA buffer (pH9. 0). Samples were incubated with primary antibody(1:500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.
  • 59 - BCKDHB Antibody (N-term) AP10672a
    Immunohistochemical analysis of paraffin-embedded Human kidney tissue using AP10672a performed on the Leica® BOND RXm. Tissue was fixed with formaldehyde at room temperature, antigen retrieval was by heat mediation with a EDTA buffer (pH9. 0). Samples were incubated with primary antibody(1:500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.
  • 3 - BCKDHB Antibody (N-term) AP10672a
    Immunofluorescent analysis of 4% paraformaldehyde-fixed, 0. 1% Triton X-100 permeabilized Hela cells labeling BCKDHB with AP10672a at 1/25 dilution, followed by Dylight® 488-conjugated goat anti-Rabbit IgG secondary antibody at 1/200 dilution (green). Immunofluorescence image showing Cytoplasm staining on Hela cell line. The nuclear counter stain is DAPI (blue).
  • 1 - BCKDHB Antibody (N-term) AP10672a
    All lanes : Anti-BCKDHB Antibody (N-term) at 1:1000 dilution Lane 1: Hela whole cell lysate Lane 2: Jurkat whole cell lysate Lane 3: Daudi whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 43 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
  • 1 - BCKDHB Antibody (N-term) AP10672a
    All lanes : Anti-BCKDHB Antibody (N-term) at 1:1000 dilution Lane 1: Hela whole cell lysate Lane 2: 293T/17 whole cell lysate Lane 3: Jurkat whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 43 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
  • 4 - BCKDHB Antibody (N-term) AP10672a
    BCKDHB Antibody (N-term) (Cat. #AP10672a) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.
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Product info
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P-Leica, IF, FC, WB, E
Primary Accession P21953
Other Accession NP_000047.1
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit Ig
Calculated MW 43122 Da
Additional info
Gene ID 594
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.
Dilution IHC-P-Leica~~1:500
IF~~1:25
WB~~1:1000
FC~~1:10~50
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.
StorageMaintain 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.
PrecautionsBCKDHB Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name BCKDHB
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.
Research Areas

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.

REFERENCES

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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