Article
Drosophila carrying pex3 or pex16 mutations are models of Zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes.
PloS one - 1 Jan 2011
Nakayama Minoru, Sato Hiroyasu, Okuda Takayuki, Fujisawa Nao, Kono Nozomu, Arai Hiroyuki, Suzuki Emiko, Umeda Masato, Ishikawa Hiroyuki O, Matsuno Kenji
Abstract excerpt
The peroxisome biogenesis disorders (PBDs) are currently difficult-to-treat multiple-organ dysfunction disorders that result from the defective biogenesis of peroxisomes. Genes encoding Peroxins, which are required for peroxisome biogenesis or functions, are known causative genes of PBDs. The human peroxin genes PEX3 or PEX16 are required for peroxisomal membrane protein targeting, and their mutations cause...
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