title: Ataxia and peripheral nerve hypomyelination in ADAM22-deficient mice.
abstract: BACKGROUND
  ADAM22 is a member of the ADAM gene family, but the fact that it is expressed only in the nervous systems makes it unique. ADAM22's sequence similarity to other ADAMs suggests it to be an integrin binder and thus to have a role in cell-cell or cell-matrix interactions. To elucidate the physiological functions of ADAM22, we employed gene targeting to generate ADAM22 knockout mice.
  
  
  RESULTS
  ADAM22-deficient mice were produced in a good accordance with the Mendelian ratio and appeared normal at birth. After one week, severe ataxia was observed, and all homozygotes died before weaning, probably due to convulsions. No major histological abnormalities were detected in the cerebral cortex or cerebellum of the homozygous mutants; however, marked hypomyelination of the peripheral nerves was observed.
  
  
  CONCLUSIONS
  The results of our study demonstrate that ADAM22 is closely involved in the correct functioning of the nervous system. Further analysis of ADAM22 will provide clues to understanding the mechanisms of human diseases such as epileptic seizures and peripheral neuropathy.
authors.affiliation: Tsukuba Research Laboratories, Eisai Co., Ltd., Tokodai 5-1-3, Tsukuba, Ibaraki, 300-2635, Japan. k-sagane@hhc.eisai.co.jp
authors.forename: Koji
authors.initials: K
authors.lastname: Sagane
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Hayakawa
authors.forename: Junko
authors.initials: J
authors.lastname: Kai
authors.forename: Tomoko
authors.initials: T
authors.lastname: Hirohashi
authors.forename: Eiki
authors.initials: E
authors.lastname: Takahashi
authors.forename: Norimasa
authors.initials: N
authors.lastname: Miyamoto
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Ino
authors.forename: Tohru
authors.initials: T
authors.lastname: Oki
authors.forename: Kazuto
authors.initials: K
authors.lastname: Yamazaki
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nagasu
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency* / Q000235:genetics / Q000502:physiology*; D000818:Animals; D001259:Ataxia / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D002478:Cells, Cultured; D005260:Female; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009413:Nerve Fibers, Myelinated / Q000378:metabolism* / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000172:deficiency* / Q000235:genetics / Q000502:physiology*; D010525:Peripheral Nerves / Q000378:metabolism / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000235:genetics / Q000378:metabolism* / Q000473:pathology
chemical_list: D009419:Nerve Tissue Proteins; D051722:ADAM Proteins; C121258:Adam22 protein, mouse