14 research outputs found
Induction of c-Jun immunoreactivity in spinal cord and brainstem neurons in a transgenic mouse model for amyotrophic lateral sclerosis
Transgenic mice carrying amyotrophic lateral sclerosis (ALS)-linked superoxide dismutase 1 (SOD1) mutations develop a motoneuron disease resembling human ALS. c-Jun is a transcription factor frequently induced in injured neurons. In this study we have examined the distribution of c-Jun-immunoreactivity in the brainstem and spinal cord of transgenic SOD1 mice with a glycine 93 alanine (G93A) mutation. In non-transgenic littermates c-Jun immunostaining was predominantly situated in motoneurons. The number of c-Jun immunoreactive motoneuron was reduced in SOD1(G93A) mice due to pronounced loss of motoneurons. In SOD1(G93A) mice, however, c-Jun-immunoreactivity was strongly induced in neurons in the intermediate zone (Rexed's laminae V-VIII and X) of the spinal cord and throughout the brainstem reticular formation. These findings are of interest since increased levels of c-jun also have been found in the intermediate zone of the spinal cord of ALS patients. Thus c-Jun may be involved in the neurodegenerative processes both in ALS and in motoneuron disease in SOD1(G93A) mice
Differential diagnosis in spinal and bulbar muscular atrophy clinical and molecular aspects
Kennedy disease is caused by an enlarged trinucleotide repeat sequence within the androgen receptor gene. We report here seven male patients with a benign motor neuron syndrome highly analogous to Kennedy disease but with a normal trinucleotide repeat
48th ENMC International workshop: Drug trials and clinical research in ALS 12-14 January 1997, Naarden, the Netherlands: workshop report
48th ENMC International workshop: Drug trials and clinical research in ALS 12-14 January 1997, Naarden, the Netherlands: workshop report
Neurofilament and glia alterations in the cerebral cortex in amyotrophic lateral sclerosis
Lymphocytic infiltration in the spinal cord of patients with amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a devastating systemic atrophy affecting the upper and lower motor neurons. The etiology is unknown, but one theory of pathogenesis supposes that the motor system is affected by abnormal immune responses. We have studied the prevalence and extent of lymphocytic infiltration, previously reported as a rare finding in the ALS spinal cord. Application of monoclonal antibodies against macrophages, T- and B-cells to spinal cords from 48 ALS patients disclosed a cellular mononuclear infiltrate in 38 specimens (79%), intense enough to be revealed by routine neuropathological techniques in 6 of them (12.5%); the remaining 10 cords (21%) exhibited no infiltrates. Since duration and clinical signs of the preceeding illness were the same in cases with and without infiltrates, we consider it unlikely that such infiltrates are entirely secondary to atrophy of the cord. As Wallerian degeneration is not accompanied by infiltrates of lymphocytes, their presence in the cord tracts of our material throws doubt on the conventional view that tract degeneration in ALS is exclusively Walleria