The changing scene of amyotrophic lateral sclerosis
New findings in ALS research are summarized, what they have taught us about this disease are discussed and issues that are still outstanding are examined.
VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death
It is indicated that VEGF is a modifier of motoneuron degeneration in human ALS and unveil a therapeutic potential of Vegfa for stressed motoneurons in mice.
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS
Two unbiased screens in Saccharomyces cerevisiae were performed and potent modifiers of DPR toxicity, including karyopherins and effectors of Ran-mediated nucleocytoplasmic transport were identified, providing insight into potential disease mechanisms and therapeutic targets.
The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis.
Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration
The results indicate that chronic vascular insufficiency and, possibly, insufficient Vegf-dependent neuroprotection lead to the select degeneration of motor neurons.
Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease
Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy
A missense mutation in the gene encoding 27-kDa small heat-shock protein B1 (HSPB1, also called HSP27) that segregates in the family with CMT2F is reported and four additional missense mutations are identified.
The phenotypic variability of amyotrophic lateral sclerosis
The phenotypic variability of ALS is reviewed and how it is reflected in familial and sporadic ALS, in the degree of upper and lower motor neuron involvement, in motor and extramotor involvement, and in the spectrum of ALS and frontotemporal dementia.