Mouse peroxisomal biogenesis factor 5 ELISA Kit | PEX5 elisa kit
Mouse Peroxisomal targeting signal 1 receptor, PEX5 ELISA Kit
NCBI and Uniprot Product Information
Uniprot Description
PEX5: Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Defects in PEX5 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. Inheritance is autosomal recessive. Defects in PEX5 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. Defects in PEX5 may be a cause of infantile Refsum disease (IRD). IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid. Belongs to the peroxisomal targeting signal receptor family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: Receptor, protein translocating; Membrane protein, integral
Cellular Component: Golgi apparatus; peroxisomal membrane; peroxisomal matrix; protein complex; membrane; mitochondrion; cell; cytoplasm; peroxisome; intracellular; cytosol
Molecular Function: small GTPase binding; protein C-terminus binding; enzyme binding; peroxisome targeting sequence binding; peroxisome matrix targeting signal-1 binding; protein N-terminus binding
Biological Process: endoplasmic reticulum organization and biogenesis; mitochondrion organization and biogenesis; isoprenoid biosynthetic process; mitochondrial membrane organization and biogenesis; neuromuscular process; neuron migration; protein import into peroxisome matrix; cellular lipid metabolic process; positive regulation of multicellular organism growth; cerebral cortex neuron differentiation; protein transport; fatty acid beta-oxidation; cerebral cortex cell migration; very-long-chain fatty acid metabolic process; transport; protein targeting to peroxisome; peroxisome organization and biogenesis; protein import into peroxisome matrix, docking; protein import into peroxisome matrix, translocation; protein import into peroxisome membrane; cell development; protein tetramerization
Research Articles on PEX5
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Product Notes
The Mouse PEX5 pex5 (Catalog #AAA9337847) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9337847 ELISA Kit recognizes Mouse PEX5. It is sometimes possible for the material contained within the vial of "peroxisomal biogenesis factor 5, ELISA Kit" 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
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