PEX19, NT (PEX19, HK33, PXF, Peroxisomal biogenesis factor 19, 33kD housekeeping protein, Peroxin-19, Peroxisomal farnesylated protein) (AP)

This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). 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. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some 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 Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants.\n\nApplications:\nSuitable for use in Western Blot, ELISA\n\nRecommended Dilution:\nELISA: 1:1,000\nWestern Blot: 1:100-500\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Do not freeze alkaline phosphatase conjugates which could result in a substantial loss of enzymatic activity. Light sensitive. Aliquots are stable for 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

039892-AP

Size

200ul

Applications

ELISA, WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to Alkaline Phosphatase.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Mohamed, S., et al. Am. J. Med. Genet. A 152A (9), 2318-2321 (2010) :\nSchmidt, F., et al. J. Biol. Chem. 285(33):25410-25417(2010)\nSchueller, N., et al. EMBO J. 29(15):2491-2500(2010)\nLiu, Y., et al. J Psychiatr Res (2010) In press :\nMatsuzono, Y., et al. J. Biol. Chem. 281(1):36-42(2006)

Additional Info

Human

Alternative Names

PEX19, HK33, PXF, Peroxisomal biogenesis factor 19, 33kD housekeeping protein, Peroxin-19, Peroxisomal farnesylated protein