Autor(es)

Medinas, Danilo B. ; Pereira, Nayrob ; Pereira, Rodolfo ; da Silva, Giovanna R. ; Santos, Victor H. S.

• Tipo de documento: Artigo Científico
• Fonte: BIOCHEMICAL SOCIETY TRANSACTIONS; v. N/A, p. 11-pg., 2025-07-28.

Resumo

The endoplasmic reticulum (ER) is a vital organelle involved in the biogenesis of membrane and secreted proteins. Proteostasis (protein homeostasis) in the ER relies on finely co-ordinated mechanisms for trans- location of polypeptides from the cytosol to the organelle lumen and membrane, introduction of coand post-translational modifications, protein folding and quality control, exportation of mature proteins and disposal of unfolded or aggregated species, besides the regulation of gene expression to adjust the proteostasis network to the cellular demands for protein biogenesis. Neurodevelopmental processes involving neurogenesis, neuronal migration and differentiation, neural circuit wiring, synaptogenesis, among others, require extensive proteome diversification and remodeling, with high fluxes through the secretory pathways constantly challenging ER proteostasis. Genetic defects affecting the different nodes of the ER proteostasis network can severely disturb neurodevelopment. Here, we compile evidence illustrating how perturbations to the different steps of protein biogenesis in the ER can lead to neurological disorders and present major questions to guide research in the field. (AU)

• Processo FAPESP: 21/06287-6 - Papel da proteína dissulfeto isomerase A3 em aprendizagem e memória
• Beneficiário: Danilo Bilches Medinas
• Modalidade de apoio: Auxílio à Pesquisa - Jovens Pesquisadores
• Processo FAPESP: 24/00316-2 - Caracterização histopatológica e bioquímica de uma linhagem de camundongos que expressam Pdia3 mutante associada à deficiência intelectual
• Beneficiário: Rodolfo da Silva Pereira
• Modalidade de apoio: Bolsas no Brasil - Mestrado
• Processo FAPESP: 24/00317-9 - Efeitos do nocaute condicional de Pdia3 durante o desenvolvimento no hipocampo de camundongos: avaliação de alterações comportamentais, histológicas e moleculares
• Beneficiário: Nayrob Pereira
• Modalidade de apoio: Bolsas no Brasil - Doutorado Direto