Stéphanie Bigou

Early Neurodegeneration Progresses Independently of Microglial Activation by Heparan Sulfate in the Brain of Mucopolysaccharidosis IIIB Mice

Jérôme Ausseil, Nathalie Desmaris, Stéphanie Bigou et al. · PLoS ONE · 2008 · 146 citations · Full text

Safe, Efficient, and Reproducible Gene Therapy of the Brain in the Dog Models of Sanfilippo and Hurler Syndromes

N. Matthew Ellinwood, Jérôme Ausseil, Nathalie Desmaris et al. · Molecular Therapy · 2010 · 137 citations · Full text

The endoplasmic reticulum-mitochondria interface is perturbed in PARK2 knockout mice and patients with PARK2 mutations

Clément Gautier, Zoi Erpapazoglou, François Mouton‐Liger et al. · Human Molecular Genetics · 2016 · 135 citations · Full text

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

Emmanuelle C. Genin, Blandine Madji Hounoum, Sylvie Bannwarth et al. · Acta Neuropathologica · 2019 · 114 citations

Mitochondrial Myopathy, Mitochondrial Defect, Mitochondrial Function +10

Modeling neuronal defects associated with a lysosomal disorder using patient-derived induced pluripotent stem cells

Thomas Lemonnier, Stéphane Blanchard, Diana Toli et al. · Human Molecular Genetics · 2011 · 89 citations · Full text