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List of works by Jonasz J. Weber

A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3.

scientific article published on 8 March 2017

Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease

scientific article published on 21 January 2020

Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).

scientific article

Calpains as novel players in the molecular pathogenesis of spinocerebellar ataxia type 17

scientific article published on 28 April 2022

Calpastatin ablation aggravates the molecular phenotype in cell and animal models of Huntington disease

scientific article published on 17 January 2018

Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice

scientific article

Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease.

scientific article

From pathways to targets: understanding the mechanisms behind polyglutamine disease.

scientific article

Generation of an induced pluripotent stem cell line from a patient with spinocerebellar ataxia type 3 (SCA3): HIHCNi002-A

scientific article published on 11 June 2018

Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3.

scientific article published on 23 February 2018

Killing Two Angry Birds with One Stone: Autophagy Activation by Inhibiting Calpains in Neurodegenerative Diseases and Beyond

scientific article published on 14 February 2019

Mitochondrial Dysfunction in Spinocerebellar Ataxia Type 3 Is Linked to VDAC1 Deubiquitination

scientific article published on 25 May 2022

Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3

scholarly article by Tina Harmuth published in January 2018

Olesoxime in neurodegenerative diseases: Scrutinising a promising drug candidate

scientific article published on 05 July 2019

Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat.

scientific article published on 21 October 2015

Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease.

scientific article

The calpain-suppressing effects of olesoxime in Huntington's disease.

scientific article published on 6 April 2016

Vulnerability of frontal brain neurons for the toxicity of expanded ataxin-3

article