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Biomedical subjects

M Tremblay

Publications and source records attributed to M Tremblay.

174 records · Page 10Linked to original sources
PubMedDate not supplied

A biochemical and ultrastructural evaluation of the type 2 Gaucher mouse.

Gaucher mice, created by targeted disruption of the glucocerebrosidase gene, are totally deficient in glucocerebrosidase and have a rapidly deteriorating clinical course analogous to the most severely affected type 2 human patients. An ultrastructural study of tissues from these mice revealed glucocerebroside accumulation in bone marrow, liver, spleen, and brain. This glycolipid had a characteristic elongated tubular structure and was contained in lysosomes, as demonstrated by colocalization with both ingested carbon particles and cathepsin D. In the central nervous system (CNS), glucocerebroside was diffusely stored in microglia cells and in brainstem and spinal cord neurons, but not in neurons of the cerebellum or cerebral cortex. This rostralcaudal pattern of neuronal lipid storage in these Gaucher mice replicates the pattern seen in type 2 human Gaucher patients and clearly demonstrates that glycosphingolipid catabolism and/or accumulation varies within different brain regions. Surprisingly, the cellular pathology of tissue from these Gaucher mice was relatively mild, and suggests that the early and rapid demise of both Gaucher mice and severely affected type 2 human neonates may be the result of both a neurotoxic metabolite, such as glucosylsphingosine, and other factors, such as skin water barrier dysfunction secondary to the absence of glucocerebrosidase activity.

Animals↗
PubMedDate not supplied

Myoblast transplantation between monozygotic twin girl carriers of Duchenne muscular dystrophy.

Monozygotic twin girls, both carriers of Duchenne muscular dystrophy, only one a severe symptomatic carrier and the other asymptomatic due to opposite lyonization, were studied. Myoblast clones were obtained from a muscle biopsy of the asymptomatic carrier. PCR amplification showed that most (94%) of these clones produced normal dystrophin mRNA. Roughly 704 million myoblasts were produced from 119 clones. These myoblasts were transplanted into the extensor carpi radialis (ECR) and in the biceps of one arm of the manifesting carrier while the other arm acted as the control. The strength of the patient was evaluated in a series of pre- and post-tests and a biopsy was obtained about 1 yr after the transplantation. The myoblast injections produced a significant force gain (12%-31%) in wrist extension but no force gain for elbow flexion. Muscle biopsies on the injected and control muscles obtained 1 yr after the injections showed only a small increase in the number of dystrophin positive fibers and the presence of numerous small type II fibers. The small beneficial effect of this transplantation cannot be attributed to immune problems, the donor and the recipient being identical twins, but may be due to a low level of spontaneous muscle regeneration.

Adolescent↗
PubMedDate not supplied

[Structuring action from using a model for planning health promotion programs].

The aim of this paper is to present results which illustrate the role of the PRECEDE-PROCEED model in the program planning process. Based on the results of an implementation evaluation of the EMPOWER software which supports health programming efforts, we have developed a metaphor to illustrate the use of the model. The following four functions of the model were identified: direction, coordination, articulation and transmission. Analysis of these functions demonstrated the structuring action of the model on planning. It would be interesting to further study how these four functions operate with or without the support of the software, to explore the influence of other health promotion models in their natural settings and to see in which way the same or other functions might be identified.

Community Health Services↗
PubMedDate not supplied

[Identification of community leaders].

Although many methods of measuring leadership have been developed in sociological studies, there are few articles on the feasibility of these methods. The goal of this study was to verify the feasibility of the "modified positional-reputational approach" developed by Nix. The study was conducted in a small community located north of Montreal. Nix's questionnaire was translated, adapted and administered to 49 key informants. Two hundred and fourteen leaders were selected. Three types of leaders were identified: the legitimizers, the effectors and the activists. Through a sociometric analysis, we established links between the different leaders and we described the power structure of the community. Despite a few shortcomings, Nix's approach was found extremely useful.

Community Participation↗