Antibody data
- Antibody Data
- Antigen structure
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- Product number
- abx004428 - Provider product page
- Provider
- Abbexa
- Product name
- Peroxisomal Biogenesis Factor 5 (PEX5) Antibody
- Antibody type
- Polyclonal
- Description
- PEX5 Antibody is a Rabbit Polyclonal antibody against PEX5. 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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 100 µl
- Concentration
- > 1 mg/ml
- Storage
- Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
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