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Search: authors:"Suzie Lefebvre"

9 papers found.
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Collagen XXV promotes myoblast fusion during myogenic differentiation and muscle formation

Fusion of myoblasts into multinucleated myofibers is crucial for skeletal muscle development and regeneration. However, the mechanisms controlling this process remain to be determined. Here we identified the involvement of a new extracellular matrix protein in myoblast fusion. Collagen XXV is a transmembrane-type collagen highly transcribed during early myogenesis when primary...

Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy

Perrine Delers Olivier Biondi Gwendoline Qu?rol L?o Houdebine Kevinee Khoobarry Fran?ois Girardet Philippe Burlet Anne- Sophie Armand Christophe Chanoine Jean-Fran?ois Bureau Fr?d?ric Charbonnier Suzie ... Lefebvre OPEN The hereditary neurodegenerative disorder spinal muscular atrophy (SMA) is characterized by the loss of spinal cord motor neurons and skeletal muscle atrophy. SMA is caused by mutations of

A new role for the calcineurin/NFAT pathway in neonatal myosin heavy chain expression via the NFATc2/MyoD complex during mouse myogenesis

Nissrine Daou Sylvie Lecolle Suzie Lefebvre Bruno della Gaspera Frdric Charbonnier Christophe Chanoine Anne-Sophie Armand 0 Centre d'Etude de la Sensori-Motricite, UMR 8194 CNRS, Universite Paris

Implication of the SMN complex in the biogenesis and steady state level of the Signal Recognition Particle

Nathalie Piazzon Florence Schlotter Suzie Lefebvre Maxime Dodre Agne` s Me reau Johann Soret Aurore Besse Martine Barkats R emy Bordonne Christiane Branlant Se verine Massenet Spinal muscular

The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy

Spinal muscular atrophy (SMA) is a common autosomal recessive neurodegenerative disease caused by reduced survival motor neuron (SMN) levels. The assembly machinery containing SMN is implicated in the biogenesis of the spliceosomal small nuclear ribonucleoproteins (snRNPs). SMN is present in both the cytoplasm and nucleus, where it transiently accumulates in subnuclear domains...

A novel association of the SMN protein with two major non-ribosomal nucleolar proteins and its implication in spinal muscular atrophy

Spinal muscular atrophy (SMA) is caused by the loss of functional survival motor neuron 1 (SMN1) protein. This ubiquitously expressed protein is a component of a novel complex immunodetected in both the cytoplasm and the nucleus, which is associated with complexes involved in mRNA splicing, ribosome biogenesis and transcription. Here, we study a mutant protein corresponding to...

The Role of the SMN Gene in Proximal Spinal Muscular Atrophy

Childhood spinal muscular atrophy (SMA) is a common recessive autosomal disorder that results in degeneration of lower motor neurons. The identification of the disease gene, Survival of Motor Neuron (SMN), was a major advance in understanding the molecular basis underlying this devastating neuromuscular disease. This finding has greatly improved the genetic counselling of SMA...