PEX13 cdna clone
PEX13 cDNA Clone
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 #AAA1268539) is a cDNA Clone and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: atggcgtccc agccgccacc tccccccaaa ccctgggaga cccgccgaat tccgggagcc ggaccgggac caggaccggg ccccactttc caatctgctg atttgggtcc tactttaatg acaagacctg gacaaccagc acttaccaga gtgcccccac ctattcttcc aaggccatca cagcagacag gaagtagcag tgtgaacact tttagacctg cttacagttc attttcttct ggatatggtg cctatggaaa ttcattttat ggaggctata gtccttatag ttatggatat aatgggctgg gctacaaccg cctccgtgta gatgatcttc cacccagtag atttgttcag caagctgaag aaagcagcag gggtgcattt cagtccattg aaagtattgt gcatgcattt gcctctgtca gtatgatgat ggatgctacc ttttcagctg tctataacag tttcagggct gtattggatg tagcaaatca cttttcccga ttgaaaatac actttacaaa agtgttttca gcttttgcat tggttaggac tatacggtat ctttacagac ggctacagcg gatgttaggt ttaagaagag gctctgagaa tgaagacctc tgggcagaga gtgaaggaac tgtggcatgc cttggtgctg aggaccgagc agctacctca gcaaaatctt ggccaatatt cttgttcttt gctgttatcc ttggtggtcc ttacctcatt tggaaactat tgtctactca cagtgatgaa gtaacagaca gcatcaactg ggcaagtggt gaggatgacc atgtagttgc cagagcagaa tatgattttg ctgccgtatc tgaagaagaa atttctttcc gggctggtga tatgctgaac ttagctctca aagaacaaca acccaaagtg cgtggttggc ttctggctag ccttgatggc caaacaacag gacttatacc tgcgaattat gtcaaaattc ttggcaaaag aaaaggtagg aaaacggtgg aatcaagtaa agtttccaag cagcaacaat cttttaccaa cccaacacta actaaaggag ccacggttgc tgattctttg gatgaacagg aagctgcctt tgaatctgtt tttgttgaaa ctaataaggt tccagttgca cctgattcca ttgggaaaga tggagaaaag caagatcttt ga. It is sometimes possible for the material contained within the vial of "PEX13, cDNA Clone" 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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