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

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product summary

company name :

US Biological

product type :

antibody

product name :

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

catalog :

039892

quantity :

200 ul

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human

product information

Catalog Number :

039892

Product wo Prefix :

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

Host :

rabbit

Product Type :

Pab

Category :

Antibodies

Size1 :

200 ul

Isotype :

IgG

Desc1 :

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. Applications: Suitable for use in Western Blot, ELISA Recommended Dilution: ELISA: 1:1,000 Western Blot: 1:100-500 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 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Calc Crossreactivity :

Alternate Name :

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

Immunogen :

PEX19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 32-61 amino acids from the N-terminal region of human PEX19.

Specificity :

Human

Purity :

Purified by Protein A affinity chromatography.

Form :

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

Concentration :

As reported

Desc2 :

Product Type: Pab Isotype: IgG Host: rabbit Source: human Concentration: As reported Form: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide. Purity: Purified by Protein A affinity chromatography. Immunogen: PEX19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 32-61 amino acids from the N-terminal region of human PEX19. Specificity: Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

Calc Applications Abbrev :

E WB

Storage Temperature :

-20°C

Reference :

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

Picture 1 File Name :

https://usbio-images.r.worldssl.net/prodimages/30/039892_1.jpg