Suppressed Nna1 gene expression in the brain of ataxic Syrian hamsters.
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.
Akita
Kenji
K
Biomedical Institute, Research Center, Hayashibara Biochemical Laboratories, Fujisaki, Okayama, Japan. k_akita@hayashibara.co.jp
Arai
Shigeyuki
S
Ohta
Tsunetaka
T
Hanaya
Toshiharu
T
Fukuda
Shigeharu
S
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
D009419:Nerve Tissue Proteins