Anti-PXF, CT (Peroxisomal Biogenesis Factor 19, Peroxisomal Farnesylated Protein, 33kD Housekeeping

The covalent attachment of prenyl lipids, such as farnesyl or geranylgeranyl groups, by specific transferases is indispensable for the cellular sorting of many proteins. Peroxisomal farnesylated protein or PXF, which localizes to the outer surface of peroxisomes, is 93% identical in sequence to human HK33, a predicted 299 amino acid protein. The ubiquitous transcription of the HK33 gene indicates its role as a housekeeping gene. At least four variants of HK33 have been identified, two of which are farnesylated in vitro. Evidence suggests that PXF is involved in the process of peroxisomal biogenesis or assembly. Complementation group J (CG-J) from patients with a peroxisome biogenesis disorder (PBD), such as Zellweger syndrome, has been identified. Human PXF expression restored peroxisomal protein import in fibroblasts from a patient with Zellweger syndrome of CG-J. This patient was homozygous for a point mutation in PXF. These results indicate that PXF is the causative gene for CG-J PBD and suggests that the C-terminal part of the PXF protein, including the CAAX homology box, is required for function. Applications: Suitable for use in ELISA and Immunohistochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:1,000, Immunohistochemistry: 1:50-1:100, Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.