PCCA Monoclonal Antibody (OTI1B10), TrueMAB™, OriGene

Mouse Monoclonal Antibody

Supplier:  OriGene TA810969

Catalog No. 50-168-3737

Description

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 fpropionic 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.

Specifications

• Antigen: PCCA
• Classification: Monoclonal
• Concentration: 1 mg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.02% sodium azide
• Gene Accession No.: P05165
• Gene Symbols: PCCA
• Immunogen: Full length human recombit protein of human PCCA produced in HEK293T cell.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG1

• Applications: Western Blot
• Clone: OTI1B10
• Conjugate: Unconjugated
• Gene: PCCA
• Gene Alias: propionyl-CoA carboxylase alpha subunit
• Host Species: Mouse
• Purification Method: Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 5095
• Content And Storage: -20° C, Avoid Freeze/Thaw Cycles
• Form: Liquid