Development and preliminary evaluation of the validity and reliability of a revised illness perception questionnaire for healthcare professionals.

Background: Diverging perceptions between individual patients with somatic diseases and their healthcare professionals might cause problems in communication and decision-making. To date, no measurement tool is available to compare the illness perceptions between these two groups. The Revised Illness Perception Questionnaire (IPQ-R) is a validated, widely used instrument in many patient populations with somatic conditions.

C-kit is important for SOD1(G93A) mouse survival independent of mast cells.

Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disease leading to progressive and lethal paralysis. The disease process is multi-factorial and is characterized by selective motor neuron degeneration. Previous work demonstrated that the local concentration of various growth factors can influence motor neuron survival and disease progression.

Beta-2 microglobulin is important for disease progression in a murine model for amyotrophic lateral sclerosis.

Beta-2 microglobulin (β2m) is an essential component of the major histocompatibility complex (MHC) class I proteins and in the nervous system β2m is predominantly expressed in motor neurons. As β2m can promote nerve regeneration, we investigated its potential role in amyotrophic lateral sclerosis (ALS) by investigating its expression level as well as the effect of genetically removing β2m on the disease process in mutant superoxide dismutase 1 (SOD1 (G93A) ) mice, a model of ALS. We observed a strong upregulation of β2m in motor neurons during the disease process and ubiquitous removal of β2m dramatically shortens the disease duration indicating that β2m plays an essential and positive role during the disease process.

Dantrolene is neuroprotective in vitro, but does not affect survival in SOD1(G⁹³A) mice.

Amyotrophic Lateral Sclerosis (ALS) is a devastating progressive neurodegenerative disease. One of the proposed disease mechanisms is excitotoxicity, in which excessive cytosolic calcium causes neuronal death. Although most calcium may originate from the extracellular space through activation of calcium-permeable AMPA receptors, we investigated in this study the contribution of endoplasmic reticulum calcium release by blocking the ryanodine receptor (RyR) using dantrolene.