PCCA Antibody

Western Blot: PCCA Antibody [H00005095-B01P] - Analysis of PCCA expression in human liver.

Western Blot: PCCA Antibody [H00005095-B01P] - Analysis of PCCA expression in U-2 OS.

Western Blot: PCCA Antibody [H00005095-B01P] - Analysis of PCCA expression in transfected 293T cell line by PCCA polyclonal antibody. Lane 1: PCCA transfected lysate(77.33 KDa). Lane 2: Non-transfected lysate.

• Reactivity: Hu, Mu, Ba
• Applications: WB
• Clonality: Polyclonal
• Host: Mouse
• Conjugate: Unconjugated

PCCA Antibody Summary

• Immunogen: PCCA (AAH00140.1, 1 a.a. - 703 a.a.) full-length human protein. MLSAALRTLKHVLYYSRQCLMVSRNLGSVGYDPNEKTFDKILVANRGEIACRVIRTCKKMGIKTVAIHSDVDASSVHVKMADEAVCVGPAPTSKSYLNMDAIMEAIKKTRAQAVHPGYGFLSENKEFARCLAAEDVVFIGPDTHAIQAMGDKIESKLLAKKAEVNTIPGFDGVVKDAEEAVRIAREIGYPVMIKASAGGGGKGMRIAWDDEETRDGFRLSSQEAASSFGDDRLLIEKFIDNPRHIEIQVLGDKHGNALWLNERECSIQRRNQKVVEEAPSIFLDAETRRAMGEQAVALARAVKYSSAGTVEFLVDSKKNFYFLEMNTRLQVEHPVTECITGLDLVQEMIRVAKGYPLRHKQADIRINGWAVECRVYAEDPYKSFGLPSIGRLSQYQEPLHLPGVRVDSGIQPGSDISIYYDPMISKLITYGSDRTEALKRMADALDNYVIRGVTHNIALLREVIINSRFVKGDISTKFLSDVYPDGFKGHMLTKSEKNQLLAIASSLFVAFQLRAQHFQENSRMPVIKPDIANWELSVKLHDKVHTVVASNNGSVFSVEVDGSKLNVTSTWNLASPLLSVSVDGTQRTVQCLSREAGGNMSIQFLGTVYKVNILTRLAAELNKFMLEKVTEDTSSVLRSPMPGVVVAVSVKPGDAVAEGQEICVIEAMKMQNSMTAGKTGTVKSVHCQAGDTVGEGDLLVELE
• Specificity: PCCA - propionyl Coenzyme A carboxylase, alpha polypeptide,
• Isotype: IgG
• Clonality: Polyclonal
• Host: Mouse
• Gene: PCCA
• Purity: Protein A purified

Applications/Dilutions

• Dilutions:
  • Western Blot
• Application Notes: Antibody reactivity against Recombinant Protein with GST tag on ELISA and WB and also on transfected lysate in WB. GST tag alone is used as a negative control.

Packaging, Storage & Formulations

• Storage: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
• Buffer: PBS (pH 7.4)
• Preservative: No Preservative
• Purity: Protein A purified

Notes

Quality control test: Antibody reactive against mammalian transfected lysate.

This product is produced by and distributed for Abnova, a company based in Taiwan.

Alternate Names for PCCA Antibody

• EC 6.4.1
• EC 6.4.1.3
• pccA complementation group
• PCCase alpha subunit
• PCCase subunit alpha
• propanoyl-CoA:carbon dioxide ligase alpha subunit
• Propanoyl-CoA:carbon dioxide ligase subunit alpha
• propionyl CoA carboxylase, alpha polypeptide
• propionyl Coenzyme A carboxylase, alpha polypeptide
• propionyl-CoA carboxylase alpha chain, mitochondrial

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. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for PCCA Antibody (H00005095-B01P)(4)

Publications using H00005095-B01P

• Anzmann AF, Pinto S, Busa V et al. Multi-omics studies in cellular models of methylmalonic acidemia and propionic acidemia reveal dysregulation of serine metabolism Biochim Biophys Acta Mol Basis Dis 2019-08-23 [PMID: 31449969] (WB, Human)
• Meierhofer D, Halbach M, ?en NE et al. Ataxin-2 (Atxn2)-Knock-Out Mice Show Branched Chain Amino Acids and Fatty Acids Pathway Alterations. Mol Cell Proteomics 2016-02-05 [PMID: 26850065]
• Majtan T, Kraus JP. Folding and activity of mutant cystathionine beta-synthase depends on the position and nature of the purification tag: Characterization of the R266K CBS mutant. Protein Expr Purif. 2012-02-08 [PMID: 22333527]
• Chandler RJ, Chandrasekaran S, Carrillo-Carrasco N et al. Adeno-associated virus serotype 8 (AAV8) Gene Transfer Rescues a Neonatal Lethal Murine Model of Propionic Acidemia. Hum Gene Ther. 2010-10-15 [PMID: 20950151]

Bioinformatics

• Gene Symbol: PCCA
• Entrez:
  • 5095(Human)
• Uniprot:
  • AAH00140.1(Human)