Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin

A reduction in Drp1-mediated fission compromises mitochondrial health in autosomal recessive spastic ataxia of Charlevoix Saguenay

(2016) Teisha Y. Bradshaw et al.   HUMAN MOLECULAR GENETICS

LAMP-2 is required for incorporating syntaxin-17 into autophagosomes and for their fusion with lysosomes

(2016) Virginie Hubert et al.   Biology Open

Powerhouse failure and oxidative damage in autosomal recessive spastic ataxia of Charlevoix-Saguenay

(2015) Chiara Criscuolo et al.   JOURNAL OF NEUROLOGY

Molecular chaperones and neuronal proteostasis

(2015) Heather L. Smith et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Vimentin filament organization and stress sensing depend on its single cysteine residue and zinc binding

(2015) Dolores Pérez-Sala et al.   Nature Communications

Sacs knockout mice present pathophysiological defects underlying autosomal recessive spastic ataxia of Charlevoix-Saguenay

(2014) Roxanne Larivière et al.   HUMAN MOLECULAR GENETICS

Proteasome Failure Promotes Positioning of Lysosomes around the Aggresome via Local Block of Microtubule-Dependent Transport

(2014) N. Zaarur et al.   MOLECULAR AND CELLULAR BIOLOGY

Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)

(2012) M. Girard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Regulation of autophagy by lysosomal positioning

(2011) Viktor I. Korolchuk et al.   Autophagy

Normal role of the low-molecular-weight neurofilament protein in mitochondrial dynamics and disruption in Charcot-Marie-Tooth disease

(2011) Benoit J. Gentil et al.   FASEB JOURNAL

Structural Basis of Defects in the Sacsin HEPN Domain Responsible for Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)

(2011) Guennadi Kozlov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Neurodegenerative-Disease-Related Protein Sacsin Is a Molecular Chaperone

(2011) John F. Anderson et al.   JOURNAL OF MOLECULAR BIOLOGY

Lysosomal positioning coordinates cellular nutrient responses

(2011) Viktor I. Korolchuk et al.   NATURE CELL BIOLOGY

Autosomal recessive spastic ataxia of Charlevoix–Saguenay: An overview

(2011) Yosr Bouhlal et al.   PARKINSONISM & RELATED DISORDERS

Regulation of vimentin intermediate filaments in endothelial cells by hypoxia

(2010) Tiegang Liu et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

The Sacsin Repeating Region (SRR): A Novel Hsp90-Related Supra-Domain Associated with Neurodegeneration

(2010) John F. Anderson et al.   JOURNAL OF MOLECULAR BIOLOGY

Chaperone networks: Tipping the balance in protein folding diseases

(2010) Cindy Voisine et al.   NEUROBIOLOGY OF DISEASE

Mutations in SACS cause atypical and late-onset forms of ARSACS

(2010) J. Baets et al.   NEUROLOGY

Protein turnover and inclusion body formation

(2009) Siddhartha Mitra et al.   Autophagy

The cellular pathways of neuronal autophagy and their implication in neurodegenerative diseases

(2009) Zhenyu Yue et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Vimentin supports mitochondrial morphology and organization

(2008) Ho Lam Tang et al.   BIOCHEMICAL JOURNAL

Structural Basis for Sorting Mechanism of p62 in Selective Autophagy

(2008) Yoshinobu Ichimura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ref(2)P, theDrosophila melanogasterhomologue of mammalian p62, is required for the formation of protein aggregates in adult brain

(2008) Ioannis P. Nezis et al.   JOURNAL OF CELL BIOLOGY