MicroRNAs as regulators of cell death mechanisms in amyotrophic lateral sclerosis

Investigating cell death mechanisms in amyotrophic lateral sclerosis using transcriptomics

Amyotrophic lateral sclerosis (ALS) is a motor neuron disease characterized by degeneration and loss of upper and lower motor neurons from the motor cortex, brainstem and spinal cord although evidence is suggesting that there is further involvement of other cell types in the surrounding tissue. Transcriptomic analysis by gene expression profiling using microarray technology has enabled the dete...

Programmed cell death in amyotrophic lateral sclerosis.

Mechanisms of neurodegeneration in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is the most common variant of motor neurone disease affecting adults that usually strikes during mid to late life. Its aetiology is still poorly understood, although a major breakthrough came with the discovery that mutations in the Cu/Zn superoxide dismutase (SOD1) gene affect approximately 20% of patients with familial ALS. Experiments using both transgenic...

Amyotrophic Lateral Sclerosis (ALS): Disease Mechanisms

Amyotrophic lateral sclerosis (ALS), known in the United States as Lou Gehrig’s disease, was first described by the famous French scientist and physician Jean-Martin Charcot in 1869. ALS, the most common adult-onset motor neuron disease, refers to a heterogeneous group of neurodegenerative disorders characterized by the selective loss of upper and lower motor neurons (specialized cells that con...

Cell cycle regulators in the neuronal death pathway of amyotrophic lateral sclerosis caused by mutant superoxide dismutase 1.

There is growing evidence for involvement of members of the cyclin-dependent kinase (Cdk) family in neurodegenerative disorders and in apoptotic death of neurons subjected to various insults. After our recent report that a deregulation of Cdk5 activity by p25 may contribute to pathogenesis of amyotrophic lateral sclerosis (ALS), we further examined the possible involvement of other Cdks in mice...