Preparation And Storage
Recommended Assay Procedures
Western blot: Please refer to http://www.bdbiosciences.com/pharmingen/protocols/Western_Blotting.shtml
Product Notices
- Since applications vary, each investigator should titrate the reagent to obtain optimal results.
- Please refer to www.bdbiosciences.com/us/s/resources for technical protocols.
- Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
- Source of all serum proteins is from USDA inspected abattoirs located in the United States.
Companion Products
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Peroxisomes, ubiquitous organelles of eukaryotic cells, are involved in a number of metabolic processes. Their formation involves membrane generation, targeting and insertion of peroxisomal membrane proteins (PMPs) into the membrane, and transport of matrix proteins across the newly formed membrane. Import of PMPs and synthesis of peroxisomal membranes may involve as many as 17 different PEX proteins. Mutation in any of 12 different Pex genes causes Zellweger syndrome (ZS), a disease characterized by loss of peroxisome biogenesis leading to severe neurologic, hepatic, and renal abnormalities. Mutations in two peroxisomal AAA ATPases, PEX1 and PEX6, are commonly associated with this and other neurological disorders. These ATPases form a complex in vitro and are required for normal import of proteins targeted to the peroxisome, as well as for maintaining the stability of PEX5, a peroxisomal receptor required for protein import. Substitution of aspartate for glycine at position 843 in PEX1 is the most common cause of peroxisome biogenesis disorders. Thus, PEX1 has an essential role in peroxisome biogenesis and mutation leads to Zwellweger syndrome-type diseases.
This antibody is routinely tested by western blot analysis. Other applications were tested at BD Biosciences Pharmingen during antibody development only or reported in the literature.
Development References (3)
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Collins CS, Gould SJ. Identification of a common PEX1 mutation in Zellweger syndrome. Hum Mutat. 1999; 14(1):45-53. (Biology). View Reference
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Geisbrecht BV, Collins CS, Reuber BE, Gould SJ. Disruption of a PEX1-PEX6 interaction is the most common cause of the neurologic disorders Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. Proc Natl Acad Sci U S A. 1998; 95(15):8630-8635. (Biology). View Reference
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Reuber BE, Germain-Lee E, Collins CS, et al. Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders. Nat Genet. 1997; 17(4):445-448. (Biology). View Reference
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Global - Refer to manufacturer's instructions for use and related User Manuals and Technical data sheets before using this products as described
Comparisons, where applicable, are made against older BD Technology, manual methods or are general performance claims. Comparisons are not made against non-BD technologies, unless otherwise noted.
For Research Use Only. Not for use in diagnostic or therapeutic procedures.
Refer to manufacturer's instructions for use and related User Manuals and Technical Data Sheets before using this product as described.
Comparisons, where applicable, are made against older BD technology, manual methods or are general performance claims. Comparisons are not made against non-BD technologies, unless otherwise noted.
