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PEX5 Mouse anti-Human, Porcine, Rat, Clone: 1C2C3, Proteintech
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Catalog No. 501736741 Shop All Proteintech Group Inc Products
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Catalog No. 50-173-6741 Supplier Proteintech Group Inc Supplier No. 663091IG150UL
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Mouse Monoclonal Antibody

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.

Specifications

Antigen PEX5
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Monoclonal
Clone 1C2C3
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene PEX5
Gene Accession No. P50542, Q2M2R8
Gene Alias Peroxin 5, Peroxisome receptor 1, PEX5, PTS1 BP, PTS1 receptor, PTS1R, PXR1
Gene Symbols Pex5
Host Species Mouse
Immunogen PEX5 Fusion Protein Ag3268
Purification Method Protein G
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 100512963, 312703, 5830
Target Species Human, Pig, Rat
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG1
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