𝔖 Bobbio Scriptorium
✦   LIBER   ✦

Riluzole attenuates spinal muscular atrophy disease progression in a mouse model

✍ Scribed by Hafedh Haddad; Carmen Cifuentes-Diaz; Audrey Miroglio; Natacha Roblot; Vandana Joshi; Judith Melki

Publisher
John Wiley and Sons
Year
2003
Tongue
English
Weight
325 KB
Volume
28
Category
Article
ISSN
0148-639X

No coin nor oath required. For personal study only.

πŸ“œ SIMILAR VOLUMES

Sensory involvement in spinal-bulbar mus
Sensory involvement in spinal-bulbar muscular atrophy (Kennedy's disease)
✍ G. Antonini; F. Gragnani; A. Romaniello; E.M. Pennisi; S. Morino; V. Ceschin; L. πŸ“‚ Article πŸ“… 2000 πŸ› John Wiley and Sons 🌐 English βš– 230 KB πŸ‘ 1 views

Spinal-bulbar muscular atrophy (SBMA) is a rare X-linked neuronopathy associated with an abnormal representation of androgen receptors in the nervous system. Standard nerve conduction and histopathological studies have disclosed the involvement of large myelinated sensory fibers in the spinal nerves

Mitochondrial dysfunction in a neural ce
Mitochondrial dysfunction in a neural cell model of spinal muscular atrophy
✍ Gyula Acsadi; Icksoo Lee; Xingli Li; Magomed Khaidakov; Alena Pecinova; Graham C πŸ“‚ Article πŸ“… 2009 πŸ› John Wiley and Sons 🌐 English βš– 255 KB

## Abstract Mutations of the survival motor neuron (SMN) gene in spinal muscular atrophy (SMA) lead to anterior horn cell death. The cause is unknown, but motor neurons depend substantially on mitochondrial oxidative phosphorylation (OxPhos) for normal function. Therefore, mitochondrial parameters

B2 attenuates polyglutamine-expanded and
B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy
✍ Isabella Palazzolo; Natalia B. Nedelsky; Caitlin E. Askew; George G. Harmison; A πŸ“‚ Article πŸ“… 2010 πŸ› John Wiley and Sons 🌐 English βš– 367 KB

## Abstract Expanded polyglutamine tracts cause neurodegeneration through a toxic gain‐of‐function mechanism. Generation of inclusions is a common feature of polyglutamine diseases and other protein misfolding disorders. Inclusion formation is likely to be a defensive response of the cell to the pr

Riluzole prolongs survival time and alte
Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease
✍ Johannes Schiefer; G. Bernhard Landwehrmeyer; Hans-Gerd LΓΌesse; Arne SprΓΌnken; C πŸ“‚ Article πŸ“… 2002 πŸ› John Wiley and Sons 🌐 English βš– 375 KB

Glutamate excitotoxicity has been suggested to contribute to the pathogenesis of Huntington's disease (HD). Riluzole is a substance with glutamate antagonistic properties that is used for neuroprotective treatment in amyotrophic lateral sclerosis and which is currently tested in clinical trials for

Dietary carnitine supplements slow disea
Dietary carnitine supplements slow disease progression in a putative mouse model for hereditary ceroid-lipofuscinosis
✍ Martin L. Katz; Laura M. Rice; Chun-Lan Gao πŸ“‚ Article πŸ“… 1997 πŸ› John Wiley and Sons 🌐 English βš– 515 KB

The childhood ceroid-lipofuscinoses are a group of autosomal recessively inherited disorders characterized by massive accumulation of autofluorescent lysosomal storage bodies in neurons as well as other cell types. The storage body accumulation is accompanied by severe degeneration of the central ne