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PEX19 Antibody

CAT: 0223-57-701Size: 400 µLDry Ice: NoHazardous: No
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CAT#:0223-57-701Size:400 µL
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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.
NCBI Gene ID
5824
Swiss Prot
P40855
Accession Number
P40855
Host
Rabbit
Reactivity
Human
Immunogen
This PEX19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 33-61 amino acids from the N-terminal region of human PEX19.
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Signal Transduction
Purification
This antibody is purified through a protein A column, followed by peptide affinity purification.
Concentration
Batch dependent
Homology
Predicted species reactivity based on immunogen sequence: Bovine, Rat
Buffer
Supplied in PBS with 0.09% (W/V) sodium azide.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Calculated Molecular Weight
33 kDa
Fragment
Rabbit Ig
Applications Notes
For WB starting dilution is: 1:1000
Symbol
PEX19
NCBI Official Name
Peroxisomal biogenesis factor 19
NCBI Organism
Homo sapiens
Background Reference 01
Mohamed, S., et al. Am. J. Med. Genet.152A (9), 2318-2321 (2010) :
Background Reference 02
Schmidt, F., et al. J. Biol. Chem. 285 (33) :25410-25417 (2010)
Background Reference 03
Schueller, N., et al. EMBO J. 29 (15) :2491-2500 (2010)
Background Reference 04
Liu, Y., et al.Psychiatr Res (2010) In press :
Other Product Names
Peroxisomal biogenesis factor 19, 33 kDa housekeeping protein, Peroxin-19, Peroxisomal farnesylated protein, PEX19, HK33, PXF
Tested Applications
WB
Protein ID
729723
Physical Properties
Liquid

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