Peroxisomal Biogenesis Factor 5 (PEX5) Antibody
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Description
Peroxisomal Biogenesis Factor 5 Antibody is a Rabbit Polyclonal antibody against Peroxisomal Biogenesis Factor 5.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Target: Peroxisomal Biogenesis Factor 5 (PEX5) Immunogen: Human PEX5. |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Antigen Affinity Chromatography. |
| Size 1 | 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB |
| Buffer | PBS, pH 7.3, containing 0.1% Sodium Azide and 50% Glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | P50542 |
| Gene ID | 5830 |
| NCBI Accession | NP_000310.2, NM_000319.4, NP_001124495.1, NM_001131023.1, NP_001124496.1, NM_001131024.1, NP_001124497.1, NM_001131025.1 |
| OMIM | 202370 |
| Background | Antibody anti-PEX5 |
| Status | RUO |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |
Descripción
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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.
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