title: Suppressed Nna1 gene expression in the brain of ataxic Syrian hamsters.
abstract: Ataxic Syrian hamsters with an autosomal recessive trait were analyzed. Homozygotes showed moderate ataxia beginning at seven to eight weeks of age. They were fertile and lived more than two years. The affected hamsters exhibited an adult-onset degeneration of cerebellar Purkinje neurons, followed by a slow, mild reduction in the density of granule cells. Northern hybridization demonstrated that expression of Nna1, the gene responsible for the Purkinje cell degeneration (pcd) phenotype, was almost negligible in the brain of homozygous hamsters. These results strongly suggest that pcd-type mutation is involved in the ataxic phenotype of mutant hamsters.
authors.affiliation: Biomedical Institute, Research Center, Hayashibara Biochemical Laboratories, Fujisaki, Okayama, Japan. k_akita@hayashibara.co.jp
authors.forename: Kenji
authors.initials: K
authors.lastname: Akita
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Arai
authors.forename: Tsunetaka
authors.initials: T
authors.lastname: Ohta
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Hanaya
authors.forename: Shigeharu
authors.initials: S
authors.lastname: Fukuda
mesh_terms: D000818:Animals; D017849:Animals, Inbred Strains; D001259:Ataxia / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D001483:Base Sequence; D015152:Blotting, Northern; D002531:Cerebellum / Q000378:metabolism* / Q000473:pathology; D006224:Cricetinae; D015870:Gene Expression; D005786:Gene Expression Regulation; D006720:Homozygote; D006801:Humans; D008251:Macaca; D008647:Mesocricetus; D051379:Mice; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000378:metabolism; D011689:Purkinje Cells / Q000378:metabolism / Q000473:pathology; D051381:Rats; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: D009419:Nerve Tissue Proteins