PEX1 Mouse, Unlabeled, Clone: 1, BD
Mouse Monoclonal Antibody
Manufacturer: BD Biosciences 611719
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.
Host Species: Mouse
Species Reactivity [for Features Main]: Human
Immunogen: Human PE1 aa. 1049-1256
|Human PEX1 aa. 1049-1256|
|Canine, Chicken, Human, Murine, Rat|
|Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide.|
|Store undiluted at -20°C.|
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