Giovanni Tamburini

Optimized AAV to express the unfolded protein response transcription factor XBP1s ameliorates Alzheimer’s disease features in mouse models

Mei Li Díaz, Giovanni Tamburini, Diego Arriagada, Natalia Poblete et autres

Abstract Proteostasis impairment at the level of the endoplasmic reticulum (ER) is a salient feature of Alzheimer’s disease (AD). The unfolded protein response (UPR) is the main pathway to cope with ER stress, where the expression of the transcription factor X-Box binding …

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Abstract 3435 IRE1/XBP1/BDNF axis role in the interaction between neurons and glioblastoma cells during brain cancer progression

Juan Francisco Silva Agüero, Ignacio Tapia-Dufey, Tapia-Dufey, Giovanni Tamburini et autres

Glioblastoma (GB) is an aggressive brain tumor that establishes functional synaptic connections with neurons, enhancing tumor progression. The mechanisms governing this neuron–tumor interaction remain poorly understood. Emerging evidence indicates that activation of the neuronal IRE1/XBP1 signaling pathway, a key branch of the …

Targeting the ER stress sensor IRE1 protects the liver from fibrosis through the downregulation of the proteostasis factor P4HB/PDIA1

Younis Hazari, Lama Habbouche, Valeria A. Garcia Lopez, Hery Urra et autres

BACKGROUND AIMS: Collagen is the main cargo of the secretory pathway, contributing to hepatic fibrogenesis due to extensive accumulation of extracellular matrix. An excess of collagen deposition is a characteristic feature of several chronic liver diseases. Collagen overproduction imposes pressure on the …

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Artificial enforcement of the unfolded protein response reduces disease features in multiple preclinical models of ALS/FTD

Vicente Valenzuela, Daniela Becerra, José I. Astorga, Matías Fuentealba et autres

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are part of a spectrum of diseases that share several causative genes, resulting in a combinatory of motor and cognitive symptoms and abnormal protein aggregation. Multiple unbiased studies have revealed that proteostasis impairment at …

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Author Reply to Peer Reviews of Targeting the ER stress sensor IRE1 protects the liver from fibrosis through the downregulation of the proteostasis factor P4HB/PDIA1

Younis Hazari, Hery Urra, Valeria A. Garcia Lopez, Lama Habbouche et autres

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The endoplasmic reticulum stress sensor IRE1 regulates collagen secretion through the enforcement of the proteostasis factor P4HB/PDIA1 contributing to liver damage and fibrosis

Younis Hazari, Hery Urra, Valeria A. Garcia Lopez, Javier Diaz et autres

Abstract Collagen is one the most abundant proteins and the main cargo of the secretory pathway, contributing to hepatic fibrosis and cirrhosis due to excessive deposition of extracellular matrix. Here we investigated the possible contribution of the unfolded protein response, the main …

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The unfolded protein response transcription factor XBP1s ameliorates Alzheimer’s disease by improving synaptic function and proteostasis

Claudia Duran‐Aniotz, Natalia Poblete, Catalina Rivera-Krstulovic, Álvaro O. Ardiles et autres

Alteration in the buffering capacity of the proteostasis network is an emerging feature of Alzheimer's disease (AD), highlighting the occurrence of endoplasmic reticulum (ER) stress. The unfolded protein response (UPR) is the main adaptive pathway to cope with protein folding stress at …

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Unfolded protein response IRE1/XBP1 signaling is required for healthy mammalian brain aging

Felipe Cabral‐Miranda, Giovanni Tamburini, Gabriela Martínez, Álvaro O. Ardiles et autres

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The unfolded protein response transcription factor XBP1s ameliorates Alzheimer’s disease by improving synaptic function and proteostasis

Claudia Duran‐Aniotz, Catalina Rivera-Krstulovic, Natalia Poblete, Álvaro O. Ardiles et autres

Abstract Alteration in the buffering capacity of the proteostasis network is an emerging feature of Alzheimer’s disease (AD), highlighting the occurrence of endoplasmic reticulum (ER) stress. The unfolded protein response (UPR) is the main adaptive pathway to cope with protein folding stress …

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Control of mammalian brain ageing by the unfolded protein response transcription factor XBP1

Felipe Cabral‐Miranda, Giovanni Tamburini, Gabriela Martínez, Danilo B. Medinas et autres

Abstract Brain ageing is the main risk factor to develop dementia and neurodegenerative diseases, associated with a decay in the buffering capacity of the proteostasis network. We investigated the significance of the unfolded protein response (UPR), a major signaling pathway to cope …

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