Western Blot (WB)
(Western blotAll lanes: PEX3 antibody at 1.24ug/ml+Mouse kidney tissueGoat polyclonal to rabbit at 1/10000 dilutionPredicted band size: 42 kDaObserved band size: 42 kDa)
Rabbit anti-Human, Mouse PEX3 Polyclonal Antibody | anti-PEX3 antibody
PEX3 Antibody
Western Blot (WB)
(Western blotAll lanes: PEX3 antibody at 1.24ug/ml+Mouse kidney tissueGoat polyclonal to rabbit at 1/10000 dilutionPredicted band size: 42 kDaObserved band size: 42 kDa)
Western Blot (WB)
(Western blotAll lanes: PEX3 antibody at 1.24ug/ml+Mouse kidney tissueGoat polyclonal to rabbit at 1/10000 dilutionPredicted band size: 42 kDaObserved band size: 42 kDa)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver tissue using MBS7044660 at dilution 1:100)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver tissue using MBS7044660 at dilution 1:100)
NCBI and Uniprot Product Information
NCBI Description
The product of this gene is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. 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. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 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 are a cause Zellweger syndrome (ZWS). [provided by RefSeq, Oct 2008]
Uniprot Description
PEX3: Involved in peroxisome biosynthesis and integrity. Assembles membrane vesicles before the matrix proteins are translocated. As a docking factor for PEX19, is necessary for the import of peroxisomal membrane proteins in the peroxisomes. Defects in PEX3 are the cause of peroxisome biogenesis disorder complementation group 12 (PBD-CG12); also known as PBD-CGG. 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 PEX3 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-3 family.
Protein type: Transporter; Membrane protein, multi-pass; Membrane protein, integral; Vesicle; Transporter, ABC family
Chromosomal Location of Human Ortholog: 6q24.2
Cellular Component: endoplasmic reticulum; integral to peroxisomal membrane; intracellular membrane-bound organelle; membrane; nucleoplasm; peroxisomal membrane; peroxisome; protein complex
Molecular Function: amino acid transmembrane transporter activity; lipid binding; protein binding; protein dimerization activity
Biological Process: peroxisome organization and biogenesis; positive regulation of defense response to virus by host; protein import into peroxisome membrane; transmembrane transport
Disease: Peroxisome Biogenesis Disorder 10a (zellweger)
Research Articles on PEX3
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Product Notes
The PEX3 pex3 (Catalog #AAA7044660) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PEX3 Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's PEX3 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC). Researchers should empirically determine the suitability of the PEX3 pex3 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. It is sometimes possible for the material contained within the vial of "PEX3, 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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