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PEX6 elisa kit :: Human peroxisomal biogenesis factor 6 ELISA Kit

Scan QR to view Datasheet Catalog #    MBS9322525 PEX6 elisa kit
Unit / Price
48-Strip-Wells  /  $470 +1 FREE 8GB USB
96-Strip-Wells  /  $680 +1 FREE 8GB USB
5x96-Strip-Wells  /  $3,100 +3 FREE 8GB USB
10x96-Strip-Wells  /  $6,095 +7 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

peroxisomal biogenesis factor 6 (PEX6), ELISA Kit

 Also Known As   

Human Peroxisome assembly factor 2, PEX6 ELISA Kit

 Product Synonym Names    Human Peroxisome assembly factor 2 (PEX6) ELISA kit; PAF-2; PAF2; PXAAA1; peroxisomal AAA-type ATPase 1; peroxisome biogenesis factor 6; peroxisomal biogenesis factor 6
 Product Gene Name   

PEX6 elisa kit

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Request for Current Manual Insert    Request Current Manual
 OMIM    614863
 3D Structure    ModBase 3D Structure for Q13608
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 Species Reactivity    Human
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 Preparation and Storage    Store all reagents at 2-8 degree C
 Product Note    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.
 Other Notes    Small volumes of PEX6 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 PEX6 purchase    MBS9322525 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the peroxisomal biogenesis factor 6 (PEX6) 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 PEX6. The ELISA analytical biochemical technique of the MBS9322525 kit is based on PEX6 antibody-PEX6 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PEX6 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PEX6. 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(%).
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NCBI/Uniprot data below describe general gene information for PEX6. It may not necessarily be applicable to this product.
 NCBI GI #    194018488
 NCBI GeneID    5190
 NCBI Accession #    NP_000278.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_000287.3 [Other Products]
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 UniProt Primary Accession #    Q13608 [Other Products]
 UniProt Secondary Accession #    Q5T8W1; Q8WYQ2; Q99476 [Other Products]
 UniProt Related Accession #    Q13608 [Other Products]
 Molecular Weight    104,061 Da
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 NCBI Official Full Name    peroxisome biogenesis factor 6
 NCBI Official Synonym Full Names    peroxisomal biogenesis factor 6
 NCBI Official Symbol    PEX6 [Similar Products]
 NCBI Official Synonym Symbols   
PAF2; PAF-2; PBD4A; PDB4B; PXAAA1
[Similar Products]
 NCBI Protein Information    peroxisome biogenesis factor 6; peroxin-6; peroxisomal-type ATPase 1; peroxisome assembly factor 2; peroxisomal AAA-type ATPase 1
 UniProt Protein Name    Peroxisome assembly factor 2
 UniProt Synonym Protein Names   
Peroxin-6; Peroxisomal biogenesis factor 6; Peroxisomal-type ATPase 1
 Protein Family    Peroxisomal ATPase
 UniProt Gene Name    PEX6 [Similar Products]
 UniProt Synonym Gene Names    PXAAA1; PAF-2 [Similar Products]
 UniProt Entry Name    PEX6_HUMAN
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 NCBI Summary for PEX6    This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. [provided by RefSeq, Oct 2008]
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 UniProt Comments for PEX6    PEX6: Involved in peroxisome biosynthesis. Required for stability of the PTS1 receptor. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Defects in PEX6 are the cause of peroxisome biogenesis disorder complementation group 4 (PBD-CG4); also known as PBD-CGC. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Defects in PEX6 are a cause of Zellweger syndrome (ZWS). ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. Belongs to the AAA ATPase family.

Protein type: Membrane protein, peripheral

Chromosomal Location of Human Ortholog: 6p21.1

Cellular Component: peroxisomal membrane; cytoplasm; peroxisome; cytosol

Molecular Function: protein C-terminus binding; protein binding; ATPase activity; protein complex binding; ATPase activity, coupled; ATP binding

Biological Process: protein stabilization; protein targeting to peroxisome; peroxisome organization and biogenesis; metabolic process; protein import into peroxisome matrix, translocation

Disease: Peroxisome Biogenesis Disorder 4b; Peroxisome Biogenesis Disorder 4a (zellweger)
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 Research Articles on PEX6    1. increased incidence of Zellweger syndrome in French-Canadians of Lac-St-Jean region caused by a PEX6 founder mutation
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 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.
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Products associated with PEX6 elisa kitPathways associated with PEX6 elisa kit
 Reference Product  PubMed Publications
 PEX1 elisa kit  >45 publications with PEX6 and PEX1
 PEX26 elisa kit  >12 publications with PEX6 and PEX26
 SMAD3 elisa kit  >1 publications with PEX6 and SMAD3
 Products by Pathway  Pathway Diagram
 Peroxisome Pathway antibodies  Peroxisome Pathway Diagram
 Peroxisome Pathway antibodies  Peroxisome Pathway Diagram
Diseases associated with PEX6 elisa kitOrgans/Tissues associated with PEX6 elisa kit
 Disease Name  Pubmed Publications
 Zellweger Syndrome Antibodies  >25 publications with PEX6 and Zellweger Syndrome
 Peroxisome biogenesis disorders Antibodies  >24 publications with PEX6 and Peroxisome biogenesis disorders
 Liver Diseases Antibodies  >15 publications with PEX6 and Liver Diseases
 Fatty Liver Antibodies  >2 publications with PEX6 and Fatty Liver
 Hepatomegaly Antibodies  >2 publications with PEX6 and Hepatomegaly
 Necrosis Antibodies  >2 publications with PEX6 and Necrosis
 Hemorrhage Antibodies  >1 publications with PEX6 and Hemorrhage
 Seizures Antibodies  >1 publications with PEX6 and Seizures
 Blood Coagulation Disorders Antibodies  >1 publications with PEX6 and Blood Coagulation Disorders
 Inflammation Antibodies  >1 publications with PEX6 and Inflammation
 Organ/Tissue Name  Pubmed Publications
 Ovary Antibodies  >10 publications with PEX6 and Ovary
 Liver Antibodies  >9 publications with PEX6 and Liver
 Skin Antibodies  >3 publications with PEX6 and Skin
 Blood Antibodies  >2 publications with PEX6 and Blood
 Brain Antibodies  >2 publications with PEX6 and Brain
 Bone Marrow Antibodies  >1 publications with PEX6 and Bone Marrow
 Mouth Antibodies  >1 publications with PEX6 and Mouth
 Bone Antibodies  >1 publications with PEX6 and Bone
 Muscle Antibodies  >1 publications with PEX6 and Muscle
 Kidney Antibodies  >1 publications with PEX6 and Kidney
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