ELISA/assay

Human Propionyl-CoA carboxylase alpha chain, mitochondrial, PCCA ELISA Kit

MBS9320731

48-Strip-Wells

470 USD

ELISA Kit

Human Propionyl-CoA carboxylase alpha chain, mitochondrial, PCCA ELISA Kit

[propionyl Coenzyme A carboxylase, alpha polypeptide]

[Human Propionyl-CoA carboxylase alpha chain; mitochondrial (PCCA) ELISA kit; RP11-151A6.1; PCCase alpha subunit; pccA complementation group; propanoyl-CoA:carbon dioxide ligase alpha subunit; propionyl-CoA carboxylase alpha chain; mitochondrial; propionyl-Coenzyme A carboxylase; ; propionyl Coenzyme A carboxylase; alpha polypeptide]

[propionyl-CoA carboxylase alpha chain, mitochondrial isoform a; Propionyl-CoA carboxylase alpha chain, mitochondrial; propionyl-CoA carboxylase alpha chain, mitochondrial; PCCase alpha subunit; pccA complementation group; propanoyl-CoA:carbon dioxide ligase alpha subunit; propionyl Coenzyme A carboxylase, alpha polypeptide; propionyl CoA carboxylase, alpha polypeptide; Propanoyl-CoA:carbon dioxide ligase subunit alpha]

[PCCA]

[PCCA; PCCA; PCCase subunit alpha]

PCCA_HUMAN

Human

No significant cross-reactivity or interference between this analyte and analogues is observed.

Store all reagents at 2-8 degree C

Samples: Human Body Fluids, Tissue Homogenates, Secretions Or Feces Samples. Assay Type: Quantitative Sandwich. Detection Range: 0.625 ng/ml-20 ng/ml. Sensitivity: 0.1 ng/ml.

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].

Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of PCBD1 (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

65506442

NP_000273.2

NM_000282.3

P05165

80,059 Da

2-oxobutanoate Degradation I Pathway (142413); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (160977); Glyoxylate And Dicarboxylate Metabolism Pathway (83002); Glyoxylate And Dicarboxylate Metabolism Pathway (383); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Mitochondrial Fatty Acid Beta-Oxidation Pathway (106122); Propanoate Metabolism Pathway (83004); Propanoate Metabolism Pathway (387); Propionyl-CoA Catabolism Pathway (160979)

The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]

PCCA: Defects in PCCA are the cause of propionic acidemia type I (PA-1). PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Ligase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; EC 6.4.1.3; Carbohydrate Metabolism - propanoate. Chromosomal Location of Human Ortholog: 13q32. Cellular Component: mitochondrial matrix; cytosol. Molecular Function: enzyme binding; metal ion binding; propionyl-CoA carboxylase activity; biotin carboxylase activity; ATP binding; biotin binding. Biological Process: fatty acid beta-oxidation; vitamin metabolic process; short-chain fatty acid catabolic process; cellular lipid metabolic process; biotin metabolic process; water-soluble vitamin metabolic process. Disease: Propionic Acidemia

48-Strip-Wells

470 USD

96-Strip-Wells

680

5x96-Strip-Wells

3100

10x96-Strip-Wells

6095