A role for the urokinase-type plasminogen activator system in amyotrophic lateral sclerosis.

There is substantial evidence, implicating extracellular matrix (ECM) regulating enzymes in the pathogenesis of motor neuron degeneration in amyotrophic lateral sclerosis (ALS). The most important ECM-degrading proteases are serine proteases (plasminogen activators, PA) and matrix metalloproteinases (MMPs). Since the role of MMPs in ALS has been addressed recently, we investigated the expression of the serine protease urokinase-type plasminogen activator (uPA) and its receptor in ALS.

Protective effect of metabotropic glutamate receptor inhibition on amyotrophic lateral sclerosis-cerebrospinal fluid toxicity in vitro.

Conflicting results have been reported concerning the toxicity of cerebrospinal fluid from patients with amyotrophic lateral sclerosis (ALS-CSF) when added to neuronal cultures. The possible toxic factor(s) and the exact mode of action (e.g.

Glial proliferation and metabotropic glutamate receptor expression in amyotrophic lateral sclerosis.

Accumulating evidence indicates that alterations in glial activation and disturbances in glial glutamate metabolism may contribute to the pathogenesis of amyotrophic lateral sclerosis (ALS). Metabotropic glutamate receptors (mGluRs) are involved in glutamate homeostasis as well as in glial proliferation. Using in situ hybridization and immunohistochemistry we found a strong upregulation of group I and group II mGluR mRNA and protein in ALS spinal cord as compared to controls (mGluR5 > mGluR1 > mGluR2/3).