A novel mouse model for ataxia-telangiectasia with a N-terminal mutation displays a behavioral defect and a low incidence of lymphoma but no increased oxidative burden.

Ataxia-telangiectasia (A-T) is a rare multi-system disorder caused by mutations in the ATM gene. Significant heterogeneity exists in the underlying genetic mutations and clinical phenotypes. A number of mouse models have been generated that harbor mutations in the distal region of the gene, and a recent study suggests the presence of residual ATM protein in the brain of one such model.

Restoration of competency to stand trial: a training program for persons with mental retardation.

This article describes the development and use of a formal training tool for restoration of competency in clients with mental retardation who are incompetent to stand trial. The program was developed at Eleanor Slater Hospital within the Rhode Island Department of Mental Health, Retardation and Hospitals. This article describes the development of The Slater Method, the training tool format, the procedure for use of the Slater Method, and the duration of treatment to restore competency to stand trial in clients with mental retardation.

The course of tardive dyskinesia and parkinsonism in psychiatric inpatients: 14-year follow-up.

Tardive dyskinesia and parkinsonism were assessed in 53 patients residing in a state psychiatric hospital in 1984 and 1998. A 4.0-point decrease in the mean Abnormal Involuntary Movement Scale score (6.

Termination-altering mutations in the second-largest subunit of yeast RNA polymerase III.

In order to identify catalytically important amino acid changes within the second-largest subunit of yeast RNA polymerase III, we mutagenized selected regions of its gene (RET1) and devised in vivo assays for both increased and decreased transcription termination by this enzyme. Using as the reporter gene a mutant SUP4-o tRNA gene that in one case terminates prematurely and in the other case fails to terminate, we screened mutagenized RET1 libraries for reduced and increased transcription termination, respectively. The gain in suppression phenotype was in both cases scored as a reduction in the accumulation of red pigment in yeast strains harboring the ade2-1 ochre mutation.

Apathy: a treatable syndrome.

Apathy occurs frequently in neuropsychiatric disorders both as a symptom of other syndromes and as a syndrome per se. Histories are presented of patients with a syndrome of apathy who showed clinically significant, sustained benefit from pharmacological treatment. Etiologies included non-Alzheimer's frontal lobe dementia, cerebral infarction, intracranial hemorrhage, alcoholism, and traumatic brain injury.