SDS-Page
((Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.)
Peroxisomal Biogenesis Factor 19 (PEX19) Active Protein | PEX19 active protein
Recombinant Human Peroxisomal Biogenesis Factor 19 (PEX19) (Active)
If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, p
SDS-Page
((Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.)
SDS-Page
((Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.)
NCBI and Uniprot Product Information
NCBI Description
This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2010]
Uniprot Description
PEX19: Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53. Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14); also known as PBD-CGJ. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: 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. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Defects in PEX19 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-19 family. 5 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, integral; Chaperone
Chromosomal Location of Human Ortholog: 1q23.2
Cellular Component: nucleoplasm; peroxisomal membrane; protein complex; intracellular membrane-bound organelle; brush border membrane; cytoplasm; integral to membrane; peroxisome; nucleus; cytosol
Molecular Function: protein binding; protein N-terminus binding; ATPase binding
Biological Process: peroxisome fission; protein stabilization; peroxisome organization and biogenesis; protein targeting to peroxisome; peroxisome membrane biogenesis; protein import into peroxisome membrane; transmembrane transport
Disease: Peroxisome Biogenesis Disorder 12a (zellweger)
Research Articles on PEX19
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Product Notes
The PEX19 pex19 (Catalog #AAA7135713) is an Active Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 2-296aa; Full Length of Mature Protein. The amino acid sequence is listed below: AAAEEGCSVG AEADRELEEL LESALDDFDK AKPSPAPPST TTAPDASGPQ KRSPGDTAKD ALFASQEKFF QELFDSELAS QATAEFEKAM KELAEEEPHL VEQFQKLSEA AGRVGSDMTS QQEFTSCLKE TLSGLAKNAT DLQNSSMSEE ELTKAMEGLG MDEGDGEGNI LPIMQSIMQN LLSKDVLYPS LKEITEKYPE WLQSHRESLP PEQFEKYQEQ HSVMCKICEQ FEAETPTDSE TTQKARFEMV LDLMQQLQDL GHPPKELAGE MPPGLNFDLD ALNLSGPPGA SGEQC. It is sometimes possible for the material contained within the vial of "Peroxisomal Biogenesis Factor 19 (PEX19), Active Protein" 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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