Western Blot (WB)
(WB Suggested Anti-PEX26 Antibody Titration: 0.2-1 ug/mlPositive Control: COLO205 cell lysatePEX26 is supported by BioGPS gene expression data to be expressed in COLO205)
Rabbit PEX26 Polyclonal Antibody | anti-PEX26 antibody
PEX26 antibody - middle region
Western Blot (WB)
(WB Suggested Anti-PEX26 Antibody Titration: 0.2-1 ug/mlPositive Control: COLO205 cell lysatePEX26 is supported by BioGPS gene expression data to be expressed in COLO205)
Western Blot (WB)
(WB Suggested Anti-PEX26 Antibody Titration: 0.2-1 ug/mlPositive Control: COLO205 cell lysatePEX26 is supported by BioGPS gene expression data to be expressed in COLO205)
Target Description: This gene belongs to the peroxin-26 gene family. It is probably required for protein import into peroxisomes. It anchors PEX1 and PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into pero
NCBI and Uniprot Product Information
NCBI Description
This gene belongs to the peroxin-26 gene family. It is probably required for protein import into peroxisomes. It anchors PEX1 and PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Defects in this gene are the cause of peroxisome biogenesis disorder complementation group 8 (PBD-CG8). 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). Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Dec 2010]
Uniprot Description
PEX26: Probably required for protein import into peroxisomes. Anchors PEX1 and PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Involved in the import of catalase and proteins containing a PTS2 target sequence, but not in import of proteins with a PTS1 target sequence. Defects in PEX26 are the cause of peroxisome biogenesis disorder complementation group 8 (PBD-CG8); also known as PBD-CGA. 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 13 distinct genetic groups as concluded from complementation studies. Defects in PEX26 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 PEX26 are a cause of adrenoleukodystrophy neonatal (NALD). NALD is a peroxisome biogenesis disorder characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation. Defects in PEX26 are a cause of infantile Refsum disease (IRD). IRD is a mild peroxisome biogenesis disorder. 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 peroxin-26 family.
Protein type: Membrane protein, integral
Chromosomal Location of Human Ortholog: 22q11.21
Cellular Component: integral to peroxisomal membrane; peroxisome
Molecular Function: protein C-terminus binding; protein binding; protein complex binding; ATPase binding
Biological Process: protein import into peroxisome matrix; protein import into peroxisome membrane
Disease: Peroxisome Biogenesis Disorder 7b; Peroxisome Biogenesis Disorder 7a (zellweger)
Research Articles on PEX26
Similar Products
Product Notes
The PEX26 pex26 (Catalog #AAA3213158) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PEX26 antibody - middle region reacts with Cow, Horse, Human, Pig and may cross-react with other species as described in the data sheet. AAA Biotech's PEX26 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the PEX26 pex26 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: Synthetic peptide located within the following region: ELVVGSAAFG EERRLDVLQA IHTARQQQKQ EHSGSEEAQK PNLEGSVSHK. It is sometimes possible for the material contained within the vial of "PEX26, 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
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
