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Rabbit Anti-PEX5 Recombinant Antibody (D7V4D) (CBMAB-CP1936-LY)

The product is antibody recognizes PEX5. The antibody D7V4D immunoassay techniques such as: WB.
See all PEX5 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
D7V4D
Antibody Isotype
IgG
Application
WB

Basic Information

Immunogen
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Phe326 of human PEX5 protein.
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
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!]

Format
Liquid
Buffer
100 µg/ml BSA, 50% glycerol
Preservative
0.02% sodium azide
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Peroxisomal Biogenesis Factor 5
Introduction
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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 of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Oct 2008]
Entrez Gene ID
Human5830
Mouse19305
Rat312703
UniProt ID
HumanP50542
MouseO09012
RatQ2M2R8
Alternative Names
Peroxisomal Biogenesis Factor 5; Peroxisome Receptor 1; Peroxisomal C-Terminal Targeting Signal Import Receptor; Peroxisomal Targeting Signal 1 Receptor; Peroxisomal Import Receptor 5; PTS1 Receptor; Peroxin-5; PTS1-BP; PTS1R;
Function
Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.
Biological Process
Cell developmentIEA:Ensembl
Cerebral cortex cell migrationIEA:Ensembl
Cerebral cortex neuron differentiationIEA:Ensembl
Endoplasmic reticulum organizationIEA:Ensembl
Fatty acid beta-oxidationIEA:Ensembl
Mitochondrial membrane organizationIEA:Ensembl
Negative regulation of protein-containing complex assemblyManual Assertion Based On ExperimentIDA:UniProtKB
Neuromuscular processIEA:Ensembl
Neuron migrationIEA:Ensembl
Positive regulation of multicellular organism growthIEA:Ensembl
Protein import into peroxisome matrixManual Assertion Based On ExperimentIMP:UniProtKB
Protein import into peroxisome matrix, dockingManual Assertion Based On ExperimentIDA:UniProtKB
Protein import into peroxisome matrix, translocationIDA:UniProtKB
Protein import into peroxisome membraneManual Assertion Based On ExperimentIMP:UniProtKB
Protein targeting to peroxisomeIDA:UniProtKB
Protein tetramerizationIDA:UniProtKB
Very long-chain fatty acid metabolic processIEA:Ensembl
Cellular Location
Cytoplasm
Peroxisome membrane
Its distribution appears to be dynamic. It is probably a cycling receptor found mainly in the cytoplasm and as well associated to the peroxisomal membrane through a docking factor (PEX13).
Involvement in disease
Peroxisome biogenesis disorder 2A (PBD2A):
A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and characterized clinically 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.
Peroxisome biogenesis disorder 2B (PBD2B):
A peroxisome biogenesis disorder that includes neonatal adrenoleukodystrophy (NALD) and infantile Refsum disease (IRD), two milder manifestations of the Zellweger disease spectrum. The clinical course of patients with the NALD and IRD presentation is variable and may include developmental delay, hypotonia, liver dysfunction, sensorineural hearing loss, retinal dystrophy and vision impairment. Children with the NALD presentation may reach their teens, while patients with the IRD presentation may reach adulthood. The clinical conditions are often slowly progressive in particular with respect to loss of hearing and vision. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid.
Rhizomelic chondrodysplasia punctata 5 (RCDP5):
A form of rhizomelic chondrodysplasia punctata, a disease characterized by severely disturbed endochondral bone formation, rhizomelic shortening of femur and humerus, vertebral disorders, dwarfism, cataract, cutaneous lesions, facial dysmorphism, and severe mental retardation with spasticity.
PTM
Monoubiquitination at Cys-11 is required for proper export from peroxisomes and recycling.
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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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