Goat PEX13 Polyclonal Antibody | anti-PEX13 antibody
Goat anti-PEX13 Antibody
Immunohistochemistry: In paraffin embedded Human Liver shows vesicular staining in the cytoplasm of the hepatocytes. Recommended concentration, 2-4ug/ml.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a peroxisomal membrane protein that binds the type 1 peroxisomal targeting signal receptor via a SH3 domain located in the cytoplasm. Mutations and deficiencies in peroxisomal protein importing and peroxisome assembly lead to peroxisomal biogenesis disorders, an example of which is Zellweger syndrome. [provided by RefSeq, Oct 2008]
Uniprot Description
PEX13: Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins. Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13); also known as PBD-CGH. PBD-CG13 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 PEX13 are a cause of adrenoleukodystrophy neonatal (NALD). NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long- chain fatty acids, adrenal insufficiency and mental retardation. Belongs to the peroxin-13 family.
Protein type: Membrane protein, integral
Chromosomal Location of Human Ortholog: 2p16.1
Cellular Component: integral to peroxisomal membrane; intracellular membrane-bound organelle; membrane; peroxisomal membrane; peroxisome
Molecular Function: protein binding
Biological Process: cerebral cortex cell migration; fatty acid alpha-oxidation; locomotory behavior; microtubule-based peroxisome localization; neuron migration; positive regulation of defense response to virus by host; protein import into peroxisome matrix, docking; suckling behavior
Disease: Peroxisome Biogenesis Disorder 11a (zellweger); Peroxisome Biogenesis Disorder 11b
Research Articles on PEX13
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Product Notes
The PEX13 pex13 (Catalog #AAA420179) is an Antibody produced from Goat and is intended for research purposes only. The product is available for immediate purchase. The Goat anti-PEX13 Antibody reacts with Tested: Human; Expected from sequence similarity: Human and may cross-react with other species as described in the data sheet. AAA Biotech's PEX13 can be used in a range of immunoassay formats including, but not limited to, Peptide ELISA (EIA), Immunohistochemistry (IHC). Peptide ELISA: Antibody detection limit dilution 1: 8000. Immunohistochemistry: In paraffin embedded Human Liver shows vesicular staining in the cytoplasm of the hepatocytes. Recommended concentration, 2-4ug/ml. Researchers should empirically determine the suitability of the PEX13 pex13 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. The amino acid sequence is listed below: DSIGKDGEKQ DL. It is sometimes possible for the material contained within the vial of "PEX13, 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
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