7 Result(s)

Article

Bicalutamide and Trehalose Ameliorate Spinal and Bulbar Muscular Atrophy Pathology in Mice

Spinal and bulbar muscular atrophy (SBMA) is characterized by motor neuron (MN) degeneration that leads to slowly progressive muscle weakness. It is considered a neuromuscular disease since muscle has a primar...

Mariarita Galbiati, Marco Meroni, Marina Boido, Matilde Cescon… in Neurotherapeutics (2023)

Article

Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that primarily affects motoneurons, while non-neuronal cells may contribute to disease onset and progression. Most ALS cases are charact...

Maria Elena Cicardi, Riccardo Cristofani, Paola Rusmini, Marco Meroni… in Scientific Reports (2018)

Article

The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two neurodegenerative diseases in which similar pathogenic mechanisms are involved. Both diseases associate to the high propensity of s...

Riccardo Cristofani, Valeria Crippa, Giulia Vezzoli… in Cell Stress and Chaperones (2018)

Article

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

Neurodegenerative diseases (NDs) are often associated with the presence of misfolded protein inclusions. The chaperone HSPB8 is upregulated in mice, the human brain and muscle structures affected during NDs pr...

Valeria Crippa, Vito G. D’Agostino, Riccardo Cristofani… in Scientific Reports (2016)

Article

Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy

Spinal and bulbar muscular atrophy (SBMA) is characterized by loss of motoneurons and sensory neurons, accompanied by atrophy of muscle cells. SBMA is due to an androgen receptor containing a polyglutamine tra...

Paola Rusmini, Maria Josefa Polanco, Riccardo Cristofani… in Scientific Reports (2015)

Article

The protein quality control system in motoneuron diseases

Angelo Poletti, Paola Rusmini, Valeria Crippa, Riccardo Cristofani… in SpringerPlus (2015)

Article

The role of dynein mediated transport in the clearance of misfolded proteins responsible for motoneuron diseases

Riccardo Cristofani, Elisa Giorgetti, Valeria Crippa… in SpringerPlus (2015)