Western Blot (WB)
(WB Suggested Anti-PEX7 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: MCF7 cell lysatePEX7 is supported by BioGPS gene expression data to be expressed in MCF7)
Rabbit PEX7 Polyclonal Antibody | anti-PEX7 antibody
PEX7 antibody - N-terminal region
Western Blot (WB)
(WB Suggested Anti-PEX7 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: MCF7 cell lysatePEX7 is supported by BioGPS gene expression data to be expressed in MCF7)
Western Blot (WB)
(WB Suggested Anti-PEX7 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: MCF7 cell lysatePEX7 is supported by BioGPS gene expression data to be expressed in MCF7)
Target Description: PEX7 binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the cytosolic receptor for the set of peroxisomal matrix enzymes targeted to the organelle by the peroxisome targeting signal 2 (PTS2). Defects in this gene cause peroxisome biogenesis disorders (PBDs), which are characterized by multiple defects in peroxisome function. There are at least 14 complementation groups for PBDs, with more than one phenotype being observed in cases falling into particular 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 have been associated with PBD complementation group 11 (PBD-CG11) disorders, rhizomelic chondrodysplasia punctata type 1 (RCDP1), and Refsum disease (RD). [provided by RefSeq, Oct 2008]
Uniprot Description
PEX7: Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import. Defects in PEX7 are the cause of peroxisome biogenesis disorder complementation group 11 (PBD-CG11). 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 PEX7 are the cause of rhizomelic chondrodysplasia punctata type 1 (RCDP1). RCDP1 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation. Defects in PEX7 are a cause of Refsum disease (RD); also known as phytanic acid oxidase deficiency. RD is clinically characterized by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life. Belongs to the WD repeat peroxin-7 family.
Chromosomal Location of Human Ortholog: 6q23.3
Cellular Component: peroxisomal matrix; peroxisome; cytosol
Molecular Function: enzyme binding; protein homodimerization activity; peroxisome matrix targeting signal-2 binding
Biological Process: fatty acid beta-oxidation; peroxisome organization and biogenesis; protein import into peroxisome matrix; neuron migration; endochondral ossification; ether lipid biosynthetic process
Disease: Peroxisome Biogenesis Disorder 9b; Refsum Disease, Classic; Rhizomelic Chondrodysplasia Punctata, Type 1
Research Articles on PEX7
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Product Notes
The PEX7 pex7 (Catalog #AAA3212554) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PEX7 antibody - N-terminal region reacts with Cow, Dog, Guinea Pig, Human, Mouse, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's PEX7 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 PEX7 pex7 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: MSAVCGGAAR MLRTPGRHGY AAEFSPYLPG RLACATAQHY GIAGCGTLLI. It is sometimes possible for the material contained within the vial of "PEX7, 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
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