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Propionyl-Coa Carboxylase Beta Chain, Mitochondrial (PCCB) ELISA Kit for Human

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS765020

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human PCCB antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human PCCB antibody was used as detection antibodies. The… More details

$405 / 48-Strip-Wells +1 FREE 8GB USB

Product Documentation

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Sample Manual Insert View PDF
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Testing Data View PDF
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Certificate of Analysis (COA) View PDF
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QC Report View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Protocol View PDF

Product Overview

Online Datasheet in PDF
Product Name
Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB), ELISA Kit
Full Product Name
Human Propionyl-CoA carboxylase beta chain, mitochondrial ELISA Kit
Product Gene Name
PCCB elisa kit
Product Synonym Names
PCCB (Propionyl-CoA carboxylase beta chain; mitochondrial)/Propanoyl-CoA:carbon dioxide ligase subunit beta/PCCase subunit beta
Species Reactivity
Human
Assay Type
Sandwich ELISA, Double Antibody
Samples
cell or tissue lysate, Other liquid samples
Detection Range
0.156-10ng/ml
Sensitivity
0.094ng/ml
Preparation and Storage
Store entire kit at 2-8C for short-term. For longer-term, please store the microplate & standard at -20C, while the remaining reagents can be stored at 2-8C
Molecular Weight
60,521 Da
OMIM
232050
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Typical Testing Data of PCCB elisa kit

Human PCCB ELISA Kit – Cat# MBS765020

Typical Standard Curve of PCCB elisa kit

Human PCCB ELISA Kit, Typical Standard Curve – Cat# MBS765020

Other Notes

Small volumes of PCCB elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Searchable Terms for PCCB purchase

MBS765020 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing PCCB. The ELISA analytical biochemical technique of the MBS765020 kit is based on PCCB antibody-PCCB antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PCCB antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PCCB. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Additional Details

NCBI/Uniprot data below describe general gene information for PCCB. It may not necessarily be applicable to this product.

NCBI GI #
295821216
NCBI GeneID
5096
NCBI Accession #
NP_001171485.1
UniProt Secondary Accession #
Q16813; Q96CX0; B7Z2Z4
UniProt Related Accession #
P05166
GenBank Nucleotide #
NM_001178014.1
NCBI Official Full Name
propionyl-CoA carboxylase beta chain, mitochondrial isoform 2
NCBI Official Synonym Full Names
propionyl-CoA carboxylase beta subunit
NCBI Official Symbol
PCCB
UniProt Protein Name
Propionyl-CoA carboxylase beta chain, mitochondrial
UniProt Synonym Protein Names
Propanoyl-CoA:carbon dioxide ligase subunit beta
Protein Family
Phycoerythrocyanin
UniProt Gene Name
PCCB
UniProt Synonym Gene Names
PCCase subunit beta
UniProt Entry Name
PCCB_HUMAN
NCBI Protein Information
propionyl-CoA carboxylase beta chain, mitochondrial

NCBI Summary for PCCB

The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
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UniProt Comments for PCCB

PCCB: Defects in PCCB are the cause of propionic acidemia type II (PA-2). PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 6.4.1.3; Ligase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate

Chromosomal Location of Human Ortholog: 3q21-q22

Cellular Component: cytosol; mitochondrial matrix; mitochondrion

Molecular Function: ATP binding; propionyl-CoA carboxylase activity

Biological Process: biotin metabolic process; cellular lipid metabolic process; fatty acid beta-oxidation; short-chain fatty acid catabolic process; vitamin metabolic process; water-soluble vitamin metabolic process

Disease: Propionic Acidemia
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Research Articles on PCCB

1. Ten propionic acidemia mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with PCCB elisa kit

 Reference Product  PubMed Publications
 PCCA elisa kit  >55 publications with PCCB and PCCA
 MUT elisa kit  >5 publications with PCCB and MUT
 MCEE elisa kit  >2 publications with PCCB and MCEE
 ACAA1 elisa kit  >1 publications with PCCB and ACAA1
 MCCC1 elisa kit  >1 publications with PCCB and MCCC1
 ACSS1 elisa kit  >1 publications with PCCB and ACSS1
 HLCS elisa kit  >1 publications with PCCB and HLCS

Pathways associated with PCCB elisa kit

 Products by Pathway  Pathway Diagram
 Biotin Transport And Metabolism Pathway antibodies  Biotin Transport And Metabolism Pathway Diagram
 Carbon Metabolism Pathway antibodies  Carbon Metabolism Pathway Diagram
 Carbon Metabolism Pathway antibodies  Carbon Metabolism Pathway Diagram
 Defective HLCS Causes Multiple Carboxylase Deficiency Pathway antibodies  Defective HLCS Causes Multiple Carboxylase Deficiency Pathway Diagram
 Defects In Biotin (Btn) Metabolism Pathway antibodies  Defects In Biotin (Btn) Metabolism Pathway Diagram
 Defects In Vitamin And Cofactor Metabolism Pathway antibodies  Defects In Vitamin And Cofactor Metabolism Pathway Diagram
 Disease Pathway antibodies  Disease Pathway Diagram
 Diseases Of Metabolism Pathway antibodies  Diseases Of Metabolism Pathway Diagram
 Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway antibodies  Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway Diagram
 Glyoxylate And Dicarboxylate Metabolism Pathway antibodies  Glyoxylate And Dicarboxylate Metabolism Pathway Diagram

Diseases associated with PCCB elisa kit

 Disease Name  Pubmed Publications
 Propionic Acidemia Antibodies  >58 publications with PCCB and Propionic Acidemia
 Vomiting Antibodies  >4 publications with PCCB and Vomiting
 Inflammation Antibodies  >4 publications with PCCB and Inflammation
 Fatty Liver Antibodies  >4 publications with PCCB and Fatty Liver
 Heart Diseases Antibodies  >3 publications with PCCB and Heart Diseases
 Disease Models, Animal Antibodies  >2 publications with PCCB and Disease Models, Animal
 Seizures Antibodies  >2 publications with PCCB and Seizures
 Renal Insufficiency Antibodies  >1 publications with PCCB and Renal Insufficiency
 Infertility, Female Antibodies  >1 publications with PCCB and Infertility, Female
 Liver Failure, Acute Antibodies  >1 publications with PCCB and Liver Failure, Acute

Organs/Tissues associated with PCCB elisa kit

 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >44 publications with PCCB and Blood
 Liver Antibodies  >9 publications with PCCB and Liver
 Heart Antibodies  >5 publications with PCCB and Heart
 Skin Antibodies  >4 publications with PCCB and Skin
 Intestine Antibodies  >2 publications with PCCB and Intestine
 Embryonic Tissue Antibodies  >2 publications with PCCB and Embryonic Tissue
 Muscle Antibodies  >2 publications with PCCB and Muscle
 Eye Antibodies  >1 publications with PCCB and Eye
 Kidney Antibodies  >1 publications with PCCB and Kidney
 Placenta Antibodies  >1 publications with PCCB and Placenta
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