Proteintech

PCCA Polyclonal Antibody

PCCA Antibody in Immunohistochemistry (Paraffin) (IHC (P))

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PCCA Antibody (21988-1-AP) in IHC (P)

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

PCCA Antibody in Immunoprecipitation (IP)

Product Image Gallery

Product Details

21988-1-AP

Applications

Tested Dilution

Publications

Western Blot (WB)

1:1,000-1:6,000

View 6 publications 6 publications

Immunohistochemistry (Paraffin) (IHC (P))

1:100-1:400

Immunocytochemistry (ICC/IF)

View 1 publication 1 publication

Immunoprecipitation (IP)

0.5-4.0 µg

Not applicable (N/A)

View 1 publication 1 publication

Product Specifications

Species Reactivity

Human, Mouse, Rat

Published species

Mouse

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

PCCA Fusion Protein Ag17271 (344-703 aa encoded by BC000140)

View immunogen

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.26 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS, pH 7.3, with 50% glycerol

Contains

0.02% sodium azide

Storage conditions

-20°C

Shipping conditions

Wet ice (domestic); Dry ice (international)

Product Specific Information

This antibody is specific to PCCA.

Immunogen sequence: LVQEMIRVA KGYPLRHKQA DIRINGWAVE CRVYAEDPYK SFGLPSIGRL SQYQEPLHLP GVRVDSGIQP GSDISIYYDP MISKLITYGS DRTEALKRMA DALDNYVIRG VTHNIALLRE VIINSRFVKG DISTKFLSDV YPDGFKGHML TKSEKNQLLA IASSLFVAFQ LRAQHFQENS RMPVIKPDIA NWELSVKLHD KVHTVVASNN GSVFSVEVDG SKLNVTSTWN LASPLLSVSV DGTQRTVQCL SREAGGNMSI QFLGTVYKVN ILTRLAAELN KFMLEKVTED TSSVLRSPMP GVVVAVSVKP GDAVAEGQEI CVIEAMKMQN SMTAGKTGTV KSVHCQAGDT VGEGDLLVEL E (344-703 aa encoded by BC000140)

Target Information

This protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o propionic acidemia type I (PA-1). PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

Product References

A coordinated multiorgan metabolic response contributes to human mitochondrial myopathy.

EMBO molecular medicine

Southwell N,Primiano G,Nadkarni V,Attarwala N,Beattie E,Miller D,Alam S,Liparulo I,Shurubor YI,Valentino ML,Carelli V,Servidei S,Gross SS,Manfredi G,Chen Q,D'Aurelio M

Mon Jul 10 00:00:00 UTC 2023

In Situ Peroxidase Labeling Followed by Mass-Spectrometry Reveals TIA1 Interactome.

Biology

Gourdomichali O,Zonke K,Kattan FG,Makridakis M,Kontostathi G,Vlahou A,Doxakis E

Fri Feb 11 00:00:00 UTC 2022

Holocarboxylase synthetase knockout is embryonic lethal in mice.

PloS one

Sadri M,Wang H,Kuroishi T,Li Y,Zempleni J

Thu Apr 14 00:00:00 UTC 2022

Metabolic perturbations mediated by propionyl-CoA accumulation in organs of mouse model of propionic acidemia.

Molecular genetics and metabolism

He W,Wang Y,Xie EJ,Barry MA,Zhang GF

Mon Nov 01 00:00:00 UTC 2021

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

Nature communications

Jiang L,Park JS,Yin L,Laureano R,Jacquinet E,Yang J,Liang S,Frassetto A,Zhuo J,Yan X,Zhu X,Fortucci S,Hoar K,Mihai C,Tunkey C,Presnyak V,Benenato KE,Lukacs CM,Martini PGV,Guey LT

Wed Oct 21 00:00:00 UTC 2020

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

The Journal of cell biology

Schatton D,Pla-Martin D,Marx MC,Hansen H,Mourier A,Nemazanyy I,Pessia A,Zentis P,Corona T,Kondylis V,Barth E,Schauss AC,Velagapudi V,Rugarli EI

Mon Mar 06 00:00:00 UTC 2017

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

The Journal of cell biology

Schatton D,Pla-Martin D,Marx MC,Hansen H,Mourier A,Nemazanyy I,Pessia A,Zentis P,Corona T,Kondylis V,Barth E,Schauss AC,Velagapudi V,Rugarli EI

Mon Mar 06 00:00:00 UTC 2017

Long-term sex-biased correction of circulating propionic acidemia disease markers by adeno-associated virus vectors.

Human gene therapy

Guenzel AJ,Collard R,Kraus JP,Matern D,Barry MA

Sun Mar 01 00:00:00 UTC 2015

Long-term sex-biased correction of circulating propionic acidemia disease markers by adeno-associated virus vectors.

Human gene therapy

Guenzel AJ,Collard R,Kraus JP,Matern D,Barry MA

Sun Mar 01 00:00:00 UTC 2015

Effects of adeno-associated virus serotype and tissue-specific expression on circulating biomarkers of propionic acidemia.

Human gene therapy

Guenzel AJ,Hillestad ML,Matern D,Barry MA

Mon Sep 01 00:00:00 UTC 2014

Effects of adeno-associated virus serotype and tissue-specific expression on circulating biomarkers of propionic acidemia.

Human gene therapy

Guenzel AJ,Hillestad ML,Matern D,Barry MA

Mon Sep 01 00:00:00 UTC 2014

Bioinformatics

Protein Aliases: 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 CoA-carboxylase alpha; Propionyl Coenzyme A carboxylase alpha polypeptide; propionyl Coenzyme A carboxylase, alpha polypeptide; Propionyl-CoA carboxylase alpha chain, mitochondrial; propionyl-coenzyme A carboxylase, alpha polypeptide; RP11-151A6.1

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Gene Aliases: C79630; PCCA

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UniProt ID: (Human) P05165, (Rat) P14882, (Mouse) Q91ZA3

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Entrez Gene ID: (Human) 5095, (Rat) 687008, (Mouse) 110821

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Function(s)

biotin carboxylase activity propionyl-CoA carboxylase activity ATP binding biotin binding enzyme binding metal ion binding nucleotide binding catalytic activity ligase activity

Process(es)

metabolic process fatty acid catabolic process cellular amino acid catabolic process biotin metabolic process short-chain fatty acid catabolic process

Performance Guarantee

If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*

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PCCA Polyclonal Antibody

PCCA Antibody (21988-1-AP) in IHC (P)

Product Details

21988-1-AP

Western Blot (WB)

Immunohistochemistry (Paraffin) (IHC (P))

Immunocytochemistry (ICC/IF)

Immunoprecipitation (IP)

Not applicable (N/A)

Species Reactivity

Published species

Host/Isotype

Class

Type

Immunogen

Conjugate

Form

Concentration

Purification

Storage buffer

Contains

Storage conditions

Shipping conditions

Product Specific Information

Target Information

Bioinformatics

Performance Guarantee

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