Motor neuronal death in sporadic amyotrophic lateral sclerosis (ALS) is not apoptotic. A comparative study of ALS and chronic aluminium chloride neurotoxicity in New …

BP He, MJ Strong - Neuropathology and applied Neurobiology, 2000 - Wiley Online Library
BP He, MJ Strong
Neuropathology and applied Neurobiology, 2000Wiley Online Library
Whether diseased motor neurones in sporadic amyotrophic lateral sclerosis (ALS) die via
apoptosis is unknown. Because this relates primarily to difficulties in utilizing post‐mortem
tissue from end‐stage disease, motor neurone degeneration in ALS spinal cord was
compared with that of a model of a chronic motor neurone degeneration. Degenerating
motor neurones in ALS, identified by ubiquitin immunoreactivity, did not demonstrate the
morphological characteristics of apoptosis and were not c‐Jun immunoreactive or TUNEL …
Whether diseased motor neurones in sporadic amyotrophic lateral sclerosis (ALS) die via apoptosis is unknown. Because this relates primarily to difficulties in utilizing post‐mortem tissue from end‐stage disease, motor neurone degeneration in ALS spinal cord was compared with that of a model of a chronic motor neurone degeneration. Degenerating motor neurones in ALS, identified by ubiquitin immunoreactivity, did not demonstrate the morphological characteristics of apoptosis and were not c‐Jun immunoreactive or TUNEL positive. A temporal analysis of spinal motor neurone death in the chronic AlCl3 neurotoxicity model of motor neurone degeneration was also undertaken. AlCl3 was administered intracisternally every 4 weeks and, at intervals of 51, 107, 156 and 267 days, evidence of apoptosis was sought by morphology, TUNEL hybridization or DNA laddering. Double‐labelling immunostudies were also performed with antibodies to either c‐Jun, ubiquitin or high molecular weight neurofilament (NFH) with TUNEL hybridization. Although significant neurone loss was evident, apoptosis was not found. These studies demonstrate a lack of apoptosis in ALS spinal motor neurones and suggest that this observation does not relate to the utilization of post‐mortem tissue in which apoptotic neurones may have been lost.
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