peroxisomal biogenesis factor 14, ELISA Kit

Rat Peroxisomal membrane protein PEX14 (PEX14) ELISA kit; MGC12767; NAPP2; Pex14p; dJ734G22.2; NF-E2 associated polypeptide 2; PTS1 receptor docking protein; peroxin-14; peroxisomal membrane anchor protein Pex14p; peroxisomal biogenesis factor 14

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of PEX14 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.

MBS9325104 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the peroxisomal biogenesis factor 14 (PEX14) 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 PEX14. The ELISA analytical biochemical technique of the MBS9325104 kit is based on PEX14 antibody-PEX14 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PEX14 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PEX14. 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(%).

40,936 Da

peroxisomal biogenesis factor 14

peroxisomal membrane protein PEX14; peroxin-14; PTS1 receptor-docking protein; peroxisomal membrane anchor protein PEX14

Peroxisomal membrane protein PEX14

PEX14_RAT

interacts with Pex5 and Pex7; plays a role in peroxisome biogenesis [RGD, Feb 2006]

PEX14: Component of the peroxisomal translocation machinery with PEX13 and PEX17. Interacts with both the PTS1 and PTS2 receptors. Binds directly to PEX17. Defects in PEX14 are the cause of peroxisome biogenesis disorder complementation group K (PBD-CGK). PBD-CGK is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: 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 (PBD- ZSS). Defects in PEX14 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 peroxin-14 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Adaptor/scaffold

Cellular Component: peroxisomal membrane; protein complex; intracellular membrane-bound organelle; membrane; nucleolus; integral to membrane; peroxisome; intracellular

Molecular Function: microtubule binding; beta-tubulin binding; protein N-terminus binding; transcription corepressor activity; receptor binding

Biological Process: peroxisome organization and biogenesis; negative regulation of transcription factor activity; protein import into peroxisome matrix; protein import into peroxisome matrix, docking; protein complex assembly; negative regulation of transcription, DNA-dependent; negative regulation of protein binding; protein import into peroxisome matrix, translocation; protein homooligomerization

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