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J Huntingtons Dis ; 3(3): 261-71, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25300330

RESUMO

BACKGROUND: The CAG triplet repeat expansion mutation in the HTT locus, which results in neurodegeneration in Huntington's disease, elongates a polyglutamine tract in huntingtin, a HEAT/HEAT-like protein that has been highly structurally conserved through evolution. In several organisms, huntingtin is necessary for proper cell-cell adhesion and normal development. OBJECTIVE: Dictyostelium discoideum huntingtin null (htt-) cells display a variety of developmental abnormalities and completely fail to acquire EDTA-resistant homotypic cell adhesion during starvation in suspension culture. METHODS: Here, we have assessed the hypothesis that htt may be a genetic interactor of csaA, a major regulator of EDTA-resistant homotypic cell adhesion in D. discoideum. Immunoblot analysis demonstrated that csaA protein expression is dysregulated in htt- cells. RESULTS: Unexpectedly, csaA overexpression, previously shown to rescue csaA- cell adhesion, failed to rescue the htt- adhesion defect. Thus, while htt was required for proper expression of the csaA protein, csaA overexpression was not sufficient to confer EDTA-resistant adhesion in the context of the htt- genetic background in contrast to parental cells. This implies a novel role for htt in conferring csaA-dependent, EDTA-resistant cell adhesion that warrants further investigation. Calcium supplementation restored both endogenous csaA protein levels and EDTA-resistant adhesion in htt- cells. CONCLUSIONS: Our data suggests the existence of an additional mechanism that overcomes the EDTA-resistant adhesion defect of htt- cells in the early development of D. discoideum.


Assuntos
Adesão Celular/fisiologia , Dictyostelium/fisiologia , Ácido Edético/farmacologia , Proteínas do Tecido Nervoso/metabolismo , Proteínas de Protozoários/metabolismo , Adesão Celular/efeitos dos fármacos , Dictyostelium/citologia
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