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Mouse Anti-PEX19 Recombinant Antibody (GT554) (CBMAB-P1477-YC)

Provided herein is a Mouse monoclonal antibody against Human Peroxisomal Biogenesis Factor 19. The antibody can be used for immunoassay techniques, such as WB, IHC-P.
See all PEX19 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
GT554
Antibody Isotype
IgG2a
Application
WB, IHC-P

Basic Information

Specificity
Human, Mouse
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Peroxisomal Biogenesis Factor 19
Introduction
PEX19 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.
Entrez Gene ID
Human5824
Mouse19298
UniProt ID
HumanP40855
MouseQ8VCI5
Alternative Names
Peroxisomal Biogenesis Factor 19; Peroxisomal Farnesylated Protein; 33 KDa Housekeeping Protein; Housekeeping Gene, 33kD; Peroxin-19; HK33; PXF;
Function
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.
Biological Process
Chaperone-mediated protein foldingManual Assertion Based On ExperimentIDA:UniProtKB
Establishment of protein localization to peroxisomeManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of lipid bindingManual Assertion Based On ExperimentIDA:UniProtKB
Peroxisome fissionManual Assertion Based On ExperimentIMP:UniProtKB
Peroxisome membrane biogenesisIDA:UniProtKB
Peroxisome organizationManual Assertion Based On ExperimentIMP:UniProtKB
Protein import into peroxisome membraneManual Assertion Based On ExperimentIDA:UniProtKB
Protein stabilizationManual Assertion Based On ExperimentIDA:UniProtKB
Protein targeting to peroxisomeManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Cytoplasm
Peroxisome membrane
Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes.
Involvement in disease
Peroxisome biogenesis disorder complementation group 14 (PBD-CG14):
A peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: 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 (PBD-ZSS).
Peroxisome biogenesis disorder 12A (PBD12A):
A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and clinically characterized by severe neurologic dysfunction with profound psychomotor retardation, severe hypotonia and neonatal seizures, craniofacial abnormalities, liver dysfunction, and biochemically by the absence of peroxisomes. Additional features include cardiovascular and skeletal defects, renal cysts, ocular abnormalities, and hearing impairment. Most severely affected individuals with the classic form of the disease (classic Zellweger syndrome) die within the first year of life.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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