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EBioMedicine ; 27: 258-274, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29269042

RESUMO

Age-related macular degeneration (AMD) is the leading cause of irreversible vision loss. The protein HtrA1 is enriched in retinal pigment epithelial (RPE) cells isolated from AMD patients and in drusen deposits. However, it is poorly understood how increased levels of HtrA1 affect the physiological function of the RPE at the intracellular level. Here, we developed hfRPE (human fetal retinal pigment epithelial) cell culture model where cells fully differentiated into a polarized functional monolayer. In this model, we fine-tuned the cellular levels of HtrA1 by targeted overexpression. Our data show that HtrA1 enzymatic activity leads to intracellular degradation of tubulin with a corresponding reduction in the number of microtubules, and consequently to an altered mechanical cell phenotype. HtrA1 overexpression further leads to impaired apical processes and decreased phagocytosis, an essential function for photoreceptor survival. These cellular alterations correlate with the AMD phenotype and thus highlight HtrA1 as an intracellular target for therapeutic interventions towards AMD treatment.


Assuntos
Polaridade Celular , Serina Peptidase 1 de Requerimento de Alta Temperatura A/metabolismo , Degeneração Macular/metabolismo , Degeneração Macular/patologia , Modelos Biológicos , Epitélio Pigmentado da Retina/metabolismo , Epitélio Pigmentado da Retina/patologia , Tubulina (Proteína)/metabolismo , Junções Aderentes/metabolismo , Adulto , Feto/metabolismo , Serina Peptidase 1 de Requerimento de Alta Temperatura A/genética , Humanos , Microtúbulos/metabolismo , Mutação/genética , Nanopartículas/química , Fagocitose , Polimerização , Agregados Proteicos , Ligação Proteica , Transcrição Gênica
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