Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver cancer using MBS7043721 at dilution 1:100)
Rabbit anti-Human PEX14 Polyclonal Antibody | anti-PEX14 antibody
PEX14 Antibody
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver cancer using MBS7043721 at dilution 1:100)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver cancer using MBS7043721 at dilution 1:100)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. [provided by RefSeq, Jul 2008]
Uniprot Description
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
Chromosomal Location of Human Ortholog: 1p36.22
Cellular Component: integral to membrane; intracellular; intracellular membrane-bound organelle; membrane; nucleolus; peroxisomal membrane; peroxisome; protein complex
Molecular Function: beta-tubulin binding; microtubule binding; protein binding; protein N-terminus binding; receptor binding; transcription corepressor activity
Biological Process: negative regulation of protein binding; negative regulation of transcription factor activity; negative regulation of transcription, DNA-dependent; peroxisome organization and biogenesis; protein complex assembly; protein homooligomerization; protein import into peroxisome matrix; protein import into peroxisome matrix, translocation
Disease: Peroxisome Biogenesis Disorder 13a (zellweger)
Research Articles on PEX14
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Product Notes
The PEX14 pex14 (Catalog #AAA7043721) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PEX14 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's PEX14 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC). Recommended dilution: IHC: 1:20-1:200. Researchers should empirically determine the suitability of the PEX14 pex14 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PEX14, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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