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Invitrogen™ PCCA Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA559693

Catalog No. PIPA559693


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Description

Description

Immunogen sequence: GSKLNVTSTW NLASPLLSVS VDGTQRTVQC LSREAGGNMS IQFLGTVYKV NILTRLAAEL NKFMLEKVTE DTSSVLRSPM PGVVVAVSVK PGDAVAEGQE ICVIEA Highest antigen sequence identity to the following orthologs: Mouse - 92%, Rat - 92%.

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.
TRUSTED_SUSTAINABILITY
Specifications

Specifications

PCCA
Polyclonal
Unconjugated
PCCA
C79630; Pcca; 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-CoA carboxylase alpha subunit; propionyl-Coenzyme A carboxylase, alpha polypeptide; RP11-151A6.1
Rabbit
Antigen affinity chromatography
RUO
5095
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Liquid
Immunohistochemistry (Paraffin)
0.05 mg/mL
PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
P05165
PCCA
Recombinant protein corresponding to Human PCCA. Recombinant protein control fragment (Product #RP-97988).
100 μL
Primary
Human
Antibody
IgG
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