A highly conserved protein family interacting with the fragile X mental retardation protein (FMRP) and displaying selective interactions with FMRP-related proteins FXR1P and FXR2P

The absence of the fragile X mental retardation protein (FMRP), encoded by the FMR1 gene, is responsible for pathologic manifestations in the Fragile X Syndrome, the most frequent cause of inherited mental retardation. FMRP is an RNA-binding protein associated with polysomes as part of a messenger ribonucleoprotein (mRNP) complex. Although its function is poorly understood, various observations suggest a role in local protein translation at neuronal dendrites and in dendritic spine maturation. We present here the identification of CYFIP1/2 (Cytoplasmic FMRP Interacting Proteins) as FMRP interactors. CYFIP1/2 share 88% amino acid sequence identity and represent the two members in humans of a highly conserved protein family. Remarkably, whereas CYFIP2 also interacts with the FMRP-related proteins FXR1P/2P, CYFIP1 interacts exclusively with FMRP. FMRP–CYFIP interaction involves the domain of FMRP also mediating homo- and heteromerization, thus suggesting a competition between interaction among the FXR proteins and interaction with CYFIP. CYFIP1/2 are proteins of unknown function, but CYFIP1 has recently been shown to interact with the small GTPase Rac1, which is implicated in development and maintenance of neuronal structures. Consistent with FMRP and Rac1 localization in dendritic fine structures, CYFIP1/2 are present in synaptosomal extracts.

A proposed program for national action to combat mental retardation ; report to the President.

Mode of access: Internet.

Assessment of behavior problems in persons with mental retardation living in the community : report on a workshop, September 9, 1988 /

Mode of access: Internet.

Mental retardation : plans for the future : public awareness, prevention, family and community service, systems simplification, full citizenship, international activities.

"15th annual report of the President's Committee on Mental Retardation."--T.P. verso.

Mental retardation : prevention strategies that work /

"Twelfth annual report"--Prelim. p.

Assessment of the national effort to combat mental retardation from biomedical causes : conference proceedings, November 14-16, 1983, Washington, D.C.

Item 529-B-8

Mental retardation : activities of the U.S. Department of Health, Education, and Welfare.

Description based on: 1962.

Compilation of selected public health laws : including particularly the Public Health Service Act, the Clean Air Act, the Solid Waste Disposal Act, the Mental Retardation Facilities and Community Mental Health Centers Construction Act, and the Federal Water Pollution Control Act.

Mode of access: Internet.

Compilation of selected public health laws: including particularly the Public health service act, the Clean air act, the Solid waste disposal act, the Mental retardation facilities and community mental health centers construction act, and the Narcotic addict rehabilitation act, the Federal food, drug, and cosmetic act, the Federal hazardous substances act, and the fair packaging and labeling act.

Prepared for the use of the Senate Committee on Labor and Public Welfare and the House Committee on Interstate and Foreign Commerce.

Self-esteem and job satisfaction of adults with mild mental retardation in sheltered workshops and supported employment programs

A sample of 200 adults with mild mental retardation were assessed on overall job satisfaction and self-esteem using the Vocational Program Evaluation Profile and the Coopersmith Self-esteem Inventory. The subjects worked either in a sheltered workshop or in a supported employment setting. Results indicated that there was a significant relationship between self-esteem and job satisfaction for both groups of subjects. In addition, subjects who worked in supported employment reported significantly higher levels of job satisfaction also. There was also an interaction between place of residence and place of employment when looking at self-esteem; those who live in a semi-independent home and work in supported employment reported the highest levels of self-esteem. These results are discussed in terms of the social validity of supported-employment for persons with mild mental retardation. ^

The effect of self-operated prompts on leisure participation by persons with mental retardation

The use of permanent prompts for individuals with severe disabilities has been shown to be effective in acquiring new skills, improving the accuracy and maintenance of existing skills, and in eliminating the need for trainer prompts. However, this technology has not been evaluated for its potential impact in promoting leisure participation. In this study, two men with mental retardation were administered auditory and/or visual prompts to investigate their effect on increasing participation in a leisure activity. In addition, their performance was evaluated to establish whether continuous versus intermittent schedules of the prompts would influence participation. The data were collected using a partial interval observation system and evaluated using a multiple baseline and an alternating treatment design. While both men increased their participation modestly in the leisure activity, there was no substantial difference between the two prompting schedules. ^

Examining a proposed job retention model for adult workers with mental retardation in supported employment

This quantitative study investigated the predictive relationships and interaction between factors such as work-related social behaviors (WRSB), self-determination (SD), person-job congruency (PJC), job performance (JP), job satisfaction (JS), and job retention (JR). A convenience sample of 100 working adults with MR were selected from supported employment agencies. Data were collected using a survey test battery of standardized instruments. The hypotheses were analyzed using three multiple regression analyses to identify significant relationships. Beta weights and hierarchical regression analysis determined the percentage of the predictor variables contribution to the total variance of the criterion variables, JR, JP, and JS. ^ The findings highlight the importance of self-determination skills in predicting job retention, satisfaction, and performance for employees with MR. Consistent with the literature and hypothesized model, there was a predictive relationship between SD, JS and JR. Furthermore, SD and PJC were predictors of JP. SD and JR were predictors of JS. Interestingly, the results indicated no significant relationship between JR and JP, or between JP and JS, or between PJC and JS. This suggests that there is a limited fit between the hypothesized model and the study's findings. However, the theoretical contribution made by this study is that self-determination is a particularly relevant predictor of important work outcomes including JR, JP, and JS. This finding is consistent with Deci's (1992) Self-Determination Theory and Wehmeyer's (1996) argument that SD skills in individuals with disabilities have important consequences for the success in transitioning from school to adult and work life. This study provides job retention strategies that offer rehabilitation and HR professionals a useful structure for understanding and implementing job retention interventions for people with MR. ^ The study concluded that workers with mental retardation who had more self-determination skills were employed longer, more satisfied, and better performers on the job. Also, individuals whose jobs were matched to their interests and abilities (person-job congruency) were better at self-determination skills. ^