9 Result(s)

Article

Correction: Administration of adipose-derived stem cells extracellular vesicles in a murine model of spinal muscular atrophy: effects of a new potential therapeutic strategy

Federica Virla, Ermanna Turano, Ilaria Scambi… in Stem Cell Research & Therapy (2024)

Article

Administration of adipose-derived stem cells extracellular vesicles in a murine model of spinal muscular atrophy: effects of a new potential therapeutic strategy

Spinal Muscular Atrophy (SMA) is an autosomal-recessive neuromuscular disease affecting children. It is caused by the mutation or deletion of the survival motor neuron 1 (SMN1) gene resulting in lower motor neuro...

Federica Virla, Ermanna Turano, Ilaria Scambi… in Stem Cell Research & Therapy (2024)

Article

hESC-derived striatal progenitors grafted into a Huntington’s disease rat model support long-term functional motor recovery by differentiating, self-organizing and connecting into the lesioned striatum

Huntington’s disease (HD) is a motor and cognitive neurodegenerative disorder due to prominent loss of striatal medium spiny neurons (MSNs). Cell replacement using human embryonic stem cells (hESCs) derivative...

Roberta Schellino, Dario Besusso, Roberta Parolisi… in Stem Cell Research & Therapy (2023)

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

Moxifloxacin rescues SMA phenotypes in patient-derived cells and animal model

Spinal muscular atrophy (SMA) is a genetic disease resulting in the loss of α-motoneurons followed by muscle atrophy. It is caused by knock-out mutations in the survival of motor neuron 1 (SMN1) gene, which has a...

Camille Januel, Giovanna Menduti, Kamel Mamchaoui… in Cellular and Molecular Life Sciences (2022)

Article

Activation of the Hepcidin-Ferroportin1 pathway in the brain and astrocytic–neuronal crosstalk to counteract iron dyshomeostasis during aging

During physiological aging, iron accumulates in the brain with a preferential distribution in regions that are more vulnerable to age-dependent neurodegeneration such as the cerebral cortex and hippocampus. In...

Mariarosa Mezzanotte, Giorgia Ammirata, Marina Boido, Serena Stanga… in Scientific Reports (2022)

Article

Transplantation of clinical-grade human neural stem cells reduces neuroinflammation, prolongs survival and delays disease progression in the SOD1 rats

Stem cells are emerging as a therapeutic option for incurable diseases, such as Amyotrophic Lateral Sclerosis (ALS). However, critical issues are related to their origin as well as to the need to deepen our kn...

Cristina Zalfa, Laura Rota Nodari, Elena Vacchi, Maurizio Gelati… in Cell Death & Disease (2019)

Article

Hyperactivity of Rac1-GTPase pathway impairs neuritogenesis of cortical neurons by altering actin dynamics

The small-GTPase Rac1 is a key molecular regulator linking extracellular signals to actin cytoskeleton dynamics. Loss-of-function mutations in RAC1 and other genes of the Rac signaling pathway have been implic...

Valentina Zamboni, Maria Armentano, Gaia Berto, Elisa Ciraolo… in Scientific Reports (2018)

Article

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

Spinal muscular atrophy (SMA) is a recessive autosomal neuromuscular disease, due to homozygous mutations or deletions in the telomeric survival motoneuron gene 1 (SMN1). SMA is characterized by motor impairment,...

Antonio Piras, Lorenzo Schiaffino, Marina Boido, Valeria Valsecchi… in Cell Death & Disease (2017)