PEX1 Pure 1/PEX 150ug

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.

1.Since applications vary, each investigator should titrate the reagent to obtain optimal results.

2.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.