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Cat. Number:

bs-12623R-Cy7

Quantity size:

100ul

Concentration:

1mg/ml  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

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. [provided by RefSeq, Aug 2010]

Also known as:

33 kDa housekeeping protein; D1S2223E; HK33; Housekeeping gene 33kD; OK/SW-cl.22; PBD12A; Peroxin 19; Peroxin-19; Peroxisomal biogenesis factor 19; Peroxisomal farnesylated protein; PEX19; PEX19_HUMAN; PMP1; PMPI; PXF; PXMP1.

Specificity:

Storage:

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

Application:

Excitation spectrum: 710nm,743nm
Emission spectrum: 767nm
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.

Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.