Calpain Inhibition Increases SMN Protein in Spinal Cord Motoneurons and Ameliorates the Spinal Muscular Atrophy Phenotype in Mice

Blocking p62-dependent SMN degradation ameliorates spinal muscular atrophy disease phenotypes

(2018) Natalia Rodriguez-Muela et al.   JOURNAL OF CLINICAL INVESTIGATION

Nusinersen versus Sham Control in Infantile-Onset Spinal Muscular Atrophy

(2017) Richard S. Finkel et al.   NEW ENGLAND JOURNAL OF MEDICINE

Inhibition of autophagy delays motoneuron degeneration and extends lifespan in a mouse model of spinal muscular atrophy

(2017) Antonio Piras et al.   Cell Death & Disease

Autophagy modulators regulate survival motor neuron protein stability in motoneurons

(2016) Ambika Periyakaruppiah et al.   EXPERIMENTAL NEUROLOGY

Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy

(2016) Matthew E.R. Butchbach et al.   EXPERIMENTAL NEUROLOGY

Calpastatin inhibits motor neuron death and increases survival of hSOD1G93Amice

(2016) Mala V. Rao et al.   JOURNAL OF NEUROCHEMISTRY

CAST your vote: is calpain inhibition the answer to ALS?

(2016) Amanda L. Wright et al.   JOURNAL OF NEUROCHEMISTRY

CNS uptake of bortezomib is enhanced by P-glycoprotein inhibition: implications for spinal muscular atrophy

(2016) Emily Foran et al.   NEUROBIOLOGY OF DISEASE

Neuroprotective Effect of Non-viral Gene Therapy Treatment Based on Tetanus Toxin C-fragment in a Severe Mouse Model of Spinal Muscular Atrophy

(2016) Sara Oliván et al.   Frontiers in Molecular Neuroscience

Moving towards treatments for spinal muscular atrophy: hopes and limits

(2015) Brunhilde Wirth et al.   EXPERT OPINION ON EMERGING DRUGS

Disease Mechanisms and Therapeutic Approaches in Spinal Muscular Atrophy

(2015) S. Tisdale et al.   JOURNAL OF NEUROSCIENCE

Autophagy dysregulation in cell culture and animals models of spinal muscular atrophy

(2014) Sara K. Custer et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Regulation of autophagy by mTOR-dependent and mTOR-independent pathways: autophagy dysfunction in neurodegenerative diseases and therapeutic application of autophagy enhancers

(2013) Sovan Sarkar   BIOCHEMICAL SOCIETY TRANSACTIONS

Reconsider Alzheimer's disease by the ‘calpain–cathepsin hypothesis’—A perspective review

(2013) Tetsumori Yamashima   PROGRESS IN NEUROBIOLOGY

Survival motor neuron protein reduction deregulates autophagy in spinal cord motoneurons in vitro

(2013) A Garcera et al.   Cell Death & Disease

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology

(2012) Takenari Yamashita et al.   Nature Communications

Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice

(2011) Deborah Y. Kwon et al.   HUMAN MOLECULAR GENETICS

A new model to study spinal muscular atrophy: Neurite degeneration and cell death is counteracted by BCL-XL Overexpression in motoneurons

(2011) Ana Garcera et al.   NEUROBIOLOGY OF DISEASE

Altered Intracellular Ca2+ Homeostasis in Nerve Terminals of Severe Spinal Muscular Atrophy Mice

(2010) R. Ruiz et al.   JOURNAL OF NEUROSCIENCE

Molecular Determinants of Survival Motor Neuron (SMN) Protein Cleavage by the Calcium-Activated Protease, Calpain

(2010) Jennifer L. Fuentes et al.   PLoS One

Specific vulnerability of mouse spinal cord motoneurons to membrane depolarization

(2009) Myriam Gou-Fabregas et al.   JOURNAL OF NEUROCHEMISTRY

Identification of a battery of tests for drug candidate evaluation in the SMNΔ7 neonate model of spinal muscular atrophy

(2008) Bassem F. El-Khodor et al.   EXPERIMENTAL NEUROLOGY

Embryonic motor axon development in the severe SMA mouse

(2008) V. L. McGovern et al.   HUMAN MOLECULAR GENETICS

SMN complex localizes to the sarcomeric Z-disc and is a proteolytic target of calpain

(2008) Michael P. Walker et al.   HUMAN MOLECULAR GENETICS

Polyglutamine-Modulated Striatal Calpain Activity in YAC Transgenic Huntington Disease Mouse Model: Impact on NMDA Receptor Function and Toxicity

(2008) C. M. Cowan et al.   JOURNAL OF NEUROSCIENCE

Nmnat Delays Axonal Degeneration Caused by Mitochondrial and Oxidative Stress

(2008) C. Press et al.   JOURNAL OF NEUROSCIENCE

Spinal muscular atrophy

(2008) Mitchell R Lunn et al.   LANCET

Regulation of SMN Protein Stability

(2008) B. G. Burnett et al.   MOLECULAR AND CELLULAR BIOLOGY

Calpain-Mediated Signaling Mechanisms in Neuronal Injury and Neurodegeneration

(2008) P. S. Vosler et al.   MOLECULAR NEUROBIOLOGY

Novel targets for Huntington's disease in an mTOR-independent autophagy pathway

(2008) Andrea Williams et al.   Nature Chemical Biology

Plastin 3 Is a Protective Modifier of Autosomal Recessive Spinal Muscular Atrophy

(2008) G. E. Oprea et al.   SCIENCE