Publication

Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis

Publications associées (29)

NAD+ boosting reduces age-associated amyloidosis and restores mitochondrial homeostasis in muscle

Johan Auwerx, Hongbo Zhang, Hao Li, Vincenzo Sorrentino, Mario Romani, Changmyung Oh, Tanes Imamura de Lima

Aging is characterized by loss of proteostasis and mitochondrial homeostasis. Here, we provide bioinformatic evidence of dysregulation of mitochondrial and proteostasis pathways in muscle aging and diseases. Moreover, we show accumulation of amyloid-like d ...

2021

Structural strains of misfolded tau protein define different diseases

Henning Paul-Julius Stahlberg

In diseases called tauopathies, misfolded tau proteins form aggregates called fibrils. Fibrils from nine different tauopathies show that tau misfolds in many ways, enabling the diseases to be classified according to fibril structure. ...

2021

Lewy body-associated proteins: Victims, instigators, or innocent bystanders? The case of AIMP2 and alpha-synuclein

Hilal Lashuel, Salvatore Novello

Lewy bodies (LBs), one of the neuropathological defining hallmarks of Parkinson's disease (PD), are composed of a complex mixture of alpha-synuclein (aSyn) filaments and hundreds of proteins, lipids, and membranous organelles. However, these proteins' role ...

2021

To target Tau pathologies, we must embrace and reconstruct their complexities

Hilal Lashuel, Galina Limorenko

The accumulation of hyperphosphorylated fibrillar Tau aggregates in the brain is one of the defining hallmarks of Tauopathy diseases, including Alzheimer's disease. However, the primary events or molecules responsible for initiation of the pathological Tau ...

ACADEMIC PRESS INC ELSEVIER SCIENCE2021

Enforced dimerization between XBP1s and ATF6f enhances the protective effects of the UPR in models of neurodegeneration

Hilal Lashuel, Sonia Donzelli

Alteration to endoplasmic reticulum (ER) proteostasis is observed in a variety of neurodegenerative diseases associated with abnormal protein aggregation. Activation of the unfolded protein response (UPR) enables an adaptive reaction to recover ER proteost ...

2021

An integrative multi-omics approach reveals new central nervous system pathway alterations in Alzheimer's disease

Mojgan Masoodi, Christopher Clark

Background Multiple pathophysiological processes have been described in Alzheimer's disease (AD). Their inter-individual variations, complex interrelations, and relevance for clinical manifestation and disease progression remain poorly understood. We hypot ...

2021

Preclinical development of a humanized chimeric antigen receptor against B-cell maturation antigen for multiple myeloma

Jaume Bonet Martinez

Multiple myeloma is a prevalent and incurable disease, despite the development of new and effective drugs. The recent development of chimeric antigen receptor (CAR)T cells has shown impressive results in the treatment of patients with relapsed or refractor ...

FERRATA STORTI FOUNDATION2021

FTLD-TDP assemblies seed neoaggregates with subtype-specific features via a prion-like cascade

Henning Paul-Julius Stahlberg, Julien Weber, Amanda Jennifer Lewis, Marta Di Fabrizio

Morphologically distinct TDP-43 aggregates occur in clinically different FTLD-TDP subtypes, yet the mechanism of their emergence and contribution to clinical heterogeneity are poorly understood. Several lines of evidence suggest that pathological TDP-43 fo ...

WILEY2021

A New Chemoenzymatic Semisynthetic Approach Provides Insight into the Role of Phosphorylation beyond Exon1 of Huntingtin and Reveals N-Terminal Fragment Length-Dependent Distinct Mechanisms of Aggregation

Hilal Lashuel, Andreas Reif, Jonathan Jean-Pierre Ricci, Gopinath Pushparathinam, Rajasekhar Kolla, Iman Rostami

Huntington’s disease is a neurodegenerative dis- order caused by the expansion of a polyglutamine repeat (>36Q) in the N-terminal domain of the huntingtin protein (Htt), which renders the protein or fragments thereof more prone to aggregate and form inclus ...

2021