drosophila carrying pex3 or pex16 mutations are models of zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes果蝇携带pex3或pex16齐薇格综合症的突变模型,以反映其症状与过氧化物酶体的缺失.pdfVIP

Drosophila Carrying Pex3 or Pex16 Mutations Are Models of Zellweger Syndrome That Reflect Its Symptoms Associated with the Absence of Peroxisomes 1,2,3 2 2 2 4 4 Minoru Nakayama , Hiroyasu Sato , Takayuki Okuda , Nao Fujisawa , Nozomu Kono , Hiroyuki Arai , 5 6 1 1,2,3 Emiko Suzuki , Masato Umeda , Hiroyuki O. Ishikawa , Kenji Matsuno * 1 Genome and Drug Research Center, Tokyo University of Science, Noda, Chiba, Japan, 2 Department of Biological Science and Technology, Tokyo University of Science, Noda, Chiba, Japan, 3 Research Institute for Science and Technology, Tokyo University of Science, Noda, Chiba, Japan, 4 Graduate School of Pharmaceutical Science, University of Tokyo, Hongo, Bunkyo-ku, Tokyo, Japan, 5 Structural Biology Center, National Institute of Genetics, and Department of Genetics, The Graduate University for Advanced Studies, Mishima, Shizuoka, Japan, 6 Institute for Chemical Research, Kyoto University, Uji, Kyoto, Japan Abstract 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 Zellweger syndrome, a class of PBDs. Lack of understanding about the pathogenesis of Zellweger syndrome has hindered the development of effective t