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>   首页   >   产品   >   一抗   >   代谢   >   PCCA Antibody (Center)   

PCCA Antibody (Center)

Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - PCCA Antibody (Center) AP12328c
    PCCA Antibody (Center) (Cat. #AP12328c) western blot analysis in A549 cell line lysates (35ug/lane).This demonstrates the PCCA antibody detected the PCCA protein (arrow).
  • 14 - PCCA Antibody (Center) AP12328c
    PCCA Antibody (Center) (Cat. #AP12328c)immunohistochemistry analysis in formalin fixed and paraffin embedded human breast tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of PCCA Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.
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Product Information
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
WB, IHC-P, E
Primary Accession P05165
Other Accession NP_001121164.1, NP_000273.2
Reactivity Human, Rat, Mouse
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 80059 Da
Antigen Region 362-390 aa
Additional Information
Gene ID 5095
Other Names Propionyl-CoA carboxylase alpha chain, mitochondrial, PCCase subunit alpha, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCA
Target/Specificity This PCCA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 362-390 amino acids from the Central region of human PCCA.
Dilution WB~~1:1000
IHC-P~~1:100~500
E~~Use at an assay dependent concentration.
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.
PrecautionsPCCA Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name PCCA (HGNC:8653)
Function This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed:6765947, PubMed:8434582). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA (PubMed:10101253, PubMed:6765947, PubMed:8434582). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)- ethylmalonyl-CoA at a much lower rate (PubMed:6765947). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).
Cellular Location Mitochondrion matrix
Research Areas

For Research Use Only. Not For Use In Diagnostic Procedures.

BACKGROUND

The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.

REFERENCES

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Huang, C.S., et al. Nature 466(7309):1001-1005(2010)
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
MacDonald, M.J., et al. Diabetologia 52(6):1087-1091(2009)

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