Transcription Factor EB Activation Rescues Advanced αB‐Crystallin Mutation‐Induced Cardiomyopathy by Normalizing Desmin Localization

TFEB activation protects against cardiac proteotoxicity via increasing autophagic flux

(2017) Bo Pan et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

AAV9-mediated gene transfer of desmin ameliorates cardiomyopathy in desmin-deficient mice

(2016) M B Heckmann et al.   GENE THERAPY

Desmin and αB-crystallin interplay in the maintenance of mitochondrial homeostasis and cardiomyocyte survival

(2016) Antigoni Diokmetzidou et al.   JOURNAL OF CELL SCIENCE

Clinical and Mechanistic Insights Into the Genetics of Cardiomyopathy

(2016) Michael A. Burke et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases

(2016) Valeria Crippa et al.   Scientific Reports

Repetitive stimulation of autophagy-lysosome machinery by intermittent fasting preconditions the myocardium to ischemia-reperfusion injury

(2015) Rebecca J Godar et al.   Autophagy

COP9 Signalosome Controls the Degradation of Cytosolic Misfolded Proteins and Protects Against Cardiac ProteotoxicityNovelty and Significance

(2015) Huabo Su et al.   CIRCULATION RESEARCH

Neuronal-Targeted TFEB Accelerates Lysosomal Degradation of APP, Reducing A  Generation and Amyloid Plaque Pathogenesis

(2015) Q. Xiao et al.   JOURNAL OF NEUROSCIENCE

Regulation of the Transcription Factor EB-PGC1α Axis by Beclin-1 Controls Mitochondrial Quality and Cardiomyocyte Death under Stress

(2015) Xiucui Ma et al.   MOLECULAR AND CELLULAR BIOLOGY

Cardiac-Selective Expression of Extracellular Superoxide Dismutase After Systemic Injection of Adeno-Associated Virus 9 Protects the Heart Against Post–Myocardial Infarction Left Ventricular Remodeling

(2013) Prasad R. Konkalmatt et al.   Circulation-Cardiovascular Imaging

Enhanced autophagy ameliorates cardiac proteinopathy

(2013) Md. Shenuarin Bhuiyan et al.   JOURNAL OF CLINICAL INVESTIGATION

TFEB controls cellular lipid metabolism through a starvation-induced autoregulatory loop

(2013) Carmine Settembre et al.   NATURE CELL BIOLOGY

Signals from the lysosome: a control centre for cellular clearance and energy metabolism

(2013) Carmine Settembre et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The specificity of the interaction between  B-crystallin and desmin filaments and its impact on filament aggregation and cell viability

(2013) J. L. Elliott et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Enhancing lysosome biogenesis attenuates BNIP3-induced cardiomyocyte death

(2012) Xiucui Ma et al.   Autophagy

A lysosome-to-nucleus signalling mechanism senses and regulates the lysosome via mTOR and TFEB

(2012) Carmine Settembre et al.   EMBO JOURNAL

Autophagy and p62 in Cardiac Proteinopathy

(2011) Qingwen Zheng et al.   CIRCULATION RESEARCH

Atg7 Induces Basal Autophagy and Rescues Autophagic Deficiency in CryAB R120G Cardiomyocytes

(2011) J. Scott Pattison et al.   CIRCULATION RESEARCH

Characterization of the CLEAR network reveals an integrated control of cellular clearance pathways

(2011) Michela Palmieri et al.   HUMAN MOLECULAR GENETICS

Enhancement of proteasomal function protects against cardiac proteinopathy and ischemia/reperfusion injury in mice

(2011) Jie Li et al.   JOURNAL OF CLINICAL INVESTIGATION

HspB8 mutation causing hereditary distal motor neuropathy impairs lysosomal delivery of autophagosomes

(2011) Alice S. Kwok et al.   JOURNAL OF NEUROCHEMISTRY

mTORC1 Senses Lysosomal Amino Acids Through an Inside-Out Mechanism That Requires the Vacuolar H+-ATPase

(2011) R. Zoncu et al.   SCIENCE

TFEB Links Autophagy to Lysosomal Biogenesis

(2011) C. Settembre et al.   SCIENCE

Manipulation of Death Pathways in Desmin-Related Cardiomyopathy

(2010) Alina Maloyan et al.   CIRCULATION RESEARCH

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Selective degradation of aggregate-prone CryAB mutants by HSPB1 is mediated by ubiquitin–proteasome pathways

(2010) Huali Zhang et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Tragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle disease

(2009) Lev G. Goldfarb et al.   JOURNAL OF CLINICAL INVESTIGATION

Protective Effect of Geranylgeranylacetone via Enhancement of HSPB8 Induction in Desmin-Related Cardiomyopathy

(2009) Atsushi Sanbe et al.   PLoS One

A missense mutation in desmin tail domain linked to human dilated cardiomyopathy promotes cleavage of the head domain and abolishes its Z-disc localization

(2008) Manolis Mavroidis et al.   FASEB JOURNAL

Autophagy is an adaptive response in desmin-related cardiomyopathy

(2008) P. Tannous et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA