PCCA Polyclonal antibody

PCCA Polyclonal Antibody for WB, IHC, IP, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IHC, IF, IP, ELISA

Conjugate

Unconjugated

Publications

11

Cat no : 21988-1-AP

Synonyms

Propionyl-CoA carboxylase alpha chain, mitochondrial, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCase subunit alpha, EC:6.4.1.3

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Validation Data Gallery

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  WB analysis using 21988-1-AP

  Various lysates were subjected to SDS PAGE followed by western blot with 21988-1-AP (PCCA antibody) at dilution of 1:4000 incubated at room temperature for 1.5 hours.

• 

  WB analysis using 21988-1-AP

  Various lysates were subjected to SDS PAGE followed by western blot with 21988-1-AP (PCCA antibody) at dilution of 1:10000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of L02 using 21988-1-AP

  L02 cells were subjected to SDS PAGE followed by western blot with 21988-1-AP (PCCA antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

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  WB analysis of NIH/3T3 using 21988-1-AP

  NIH/3T3 cells were subjected to SDS PAGE followed by western blot with 21988-1-AP (PCCA antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis using 21988-1-AP

  Various lysates were subjected to SDS PAGE followed by western blot with 21988-1-AP (PCCA antibody) at dilution of 1:1500 incubated at room temperature for 1.5 hours.

• 

  IP experiment of L02 using 21988-1-AP

  IP result of anti-PCCA (IP:21988-1-AP, 4ug; Detection:21988-1-AP 1:1000) with L02 cells lysate 1800ug.

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  IHC staining of human liver cancer using 21988-1-AP

  Immunohistochemical analysis of paraffin-embedded human liver cancer tissue slide using 21988-1-AP (PCCA antibody) at dilution of 1:200 (under 40x lens). Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0).

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  IHC staining of human kidney using 21988-1-AP

  Immunohistochemical analysis of paraffin-embedded human kidney tissue slide using 21988-1-AP (PCCA antibody) at dilution of 1:200 (under 40x lens). Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0).

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Tested Applications

• Positive WB detected in: mouse heart tissue, L02 cells, NIH/3T3 cells, mouse liver tissue, rat liver tissue, mouse skeletal muscle tissue, rat heart tissue
• Positive IP detected in: L02 cells
• Positive IHC detected in: human liver cancer tissue, human kidney tissue; Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0

Recommended dilution

• Western Blot (WB): WB : 1:1000-1:8000
• Immunoprecipitation (IP): IP : 0.5-4.0 ug for 1.0-3.0 mg of total protein lysate
• Immunohistochemistry (IHC): IHC : 1:50-1:500

It is recommended that this reagent should be titrated in each testing system to obtain optimal results.

Sample-dependent, Check data in validation data gallery.

Published Applications

• WB: See 7 publications below
• IHC: See 1 publications below
• IF: See 2 publications below

Product Information

21988-1-AP targets PCCA in WB, IHC, IF, IP, ELISA applications and shows reactivity with human, mouse, rat samples.

• Tested Reactivity: human, mouse, rat
• Cited Reactivity: human, mouse
• Host / Isotype: Rabbit / IgG
• Class: Polyclonal
• Type: Antibody
• Immunogen: PCCA fusion protein Ag17271
• Full Name: propionyl Coenzyme A carboxylase, alpha polypeptide
• Calculated Molecular Weight: 80 kDa
• Observed Molecular Weight: 70 kDa
• GenBank Accession Number: BC000140
• Gene Symbol: PCCA
• Gene ID (NCBI): 5095
• RRID: AB_2878963
• Conjugate: Unconjugated
• Form: Liquid
• Purification Method: Antigen affinity purification
• Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
• Storage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20^oC storage. 20ul sizes contain 0.1% BSA.

Background Information

Propionyl-CoA carboxylase (PCC) catalyzes the biotin-dependent carboxylation of propionyl-CoA to D-methyl-malonyl CoA, a reaction that occurs in the mitochondrial matrix. PCC is involved in the catabolism of several essential amino acids (methionine, isoleucine, threonine and valine), as well as odd chain fatty acids and cholesterol. Deficiency of PCC results in propionic acidemia, a metabolic disorder characterized by severe metabolic ketoacidosis, vomiting, lethargy and hypotonia. PCC consists of nonidentical subunits (α and β) encoded by different genes (PCCA and PCCB, respectively). The αPCC cDNA contains an open reading frame of 2106 nucleotide bases and codes for a 702 amino acid polypeptide. The mature length subunit is 70 kDa and contains the biotin binding site.(PMID:8852656). This protein has 3 isoforms produced by alternative splicing with the molecular weight of 80 kDa, 77 kDa and 75 kDa. The full length protein has a transit peptide with 52 amino acids which will be removed in mature form. This antibody is specific to PCCA.

Publications

Species	Application	Title
human	IF	Nat Commun; Dual mRNA therapy restores metabolic function in long-term studies in mice with propionic acidemia.; Authors - Lei Jiang
mouse	WB	J Cell Biol; CLUH regulates mitochondrial metabolism by controlling translation and decay of target mRNAs.; Authors - Désirée Schatton
mouse	WB	Mol Genet Metab; Metabolic perturbations mediated by propionyl-CoA accumulation in organs of mouse model of propionic acidemia.; Authors - Wentao He
mouse	WB	Hum Gene Ther; Effects of Adeno-associated Virus Serotype and Tissue-specific Expression on Circulating Biomarkers of Propionic Acidemia.; Authors - Adam Guenzel
mouse	WB	Hum Gene Ther; Long-term Sex-Biased Correction of Circulating Propionic Acidemia Disease Markers by Adeno-Associated Virus Vectors.; Authors - Adam Guenzel
human	WB	Biology (Basel); In Situ Peroxidase Labeling Followed by Mass-Spectrometry Reveals TIA1 Interactome.; Authors - Olga Gourdomichali