PCCA Polyclonal antibody

PCCA Polyclonal Antibody for IHC, IP, WB,ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IP, IHC, IF,ELISA

Conjugate

Unconjugated

Cat no : 21988-1-AP

Synonyms

PCCA, PCCase subunit alpha



Tested Applications

Positive WB detected inmouse liver tissue, L02 cells, NIH/3T3 cells, rat liver tissue
Positive IP detected inL02 cells
Positive IHC detected inhuman 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

ApplicationDilution
Western Blot (WB)WB : 1:1000-1:6000
Immunoprecipitation (IP)IP : 0.5-4.0 ug for IP and 1:500-1:2000 for WB
Immunohistochemistry (IHC)IHC : 1:50-1:500
Sample-dependent, check data in validation data gallery

Product Information

The immunogen of 21988-1-AP is PCCA Fusion Protein expressed in E. coli.

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 numberBC000140
Gene symbol PCCA
Gene ID (NCBI) 5095
Conjugate Unconjugated
Form Liquid
Purification Method Antigen affinity purification
Storage Buffer PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Storage ConditionsStore at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.

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.

Protocols

Product Specific Protocols
WB protocol for PCCA antibody 21988-1-APDownload protocol
IHC protocol for PCCA antibody 21988-1-APDownload protocol
IP protocol for PCCA antibody 21988-1-APDownload protocol
Standard Protocols
Click here to view our Standard Protocols

Publications

SpeciesApplicationTitle
mouseWB

Hum Gene Ther

Effects of Adeno-associated Virus Serotype and Tissue-specific Expression on Circulating Biomarkers of Propionic Acidemia.

Authors - Adam Guenzel
mouseWB

Hum Gene Ther

Long-term Sex-Biased Correction of Circulating Propionic Acidemia Disease Markers by Adeno-Associated Virus Vectors.

Authors - Adam Guenzel
mouseWB

J Cell Biol

CLUH regulates mitochondrial metabolism by controlling translation and decay of target mRNAs.

Authors - Désirée Schatton
humanIF

Nat Commun

Dual mRNA therapy restores metabolic function in long-term studies in mice with propionic acidemia.

Authors - Lei Jiang
  • KD Validated