Motor neuron disease and trace element toxicity

Strong, Michael J.; He, Beiping

doi:10.1002/(sici)1520-670x(2000)13:1<131::aid-jtra14>3.0.co;2-e

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“…Because our failure to detect apoptosis may reflect the terminal stage of the disease process, a temporal analysis of motor neurone death in a model of chronic AlCl 3 neurotoxicity was undertaken. Although it is not known whether the mechanism of motor neurone death in chronic AlCl 3 ‐inoculated rabbits is exactly the same as that in ALS, there is significant overlap in the clini‐cal, neuropathological and neurochemical homology between the two processes [19,21,23]. The accumulation of phosphorylated neurofilament in the soma and axons of spinal motor neurones was again confirmed in the present study.…”

Section: Discussionsupporting

confidence: 77%

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

He¹,

Strong

2000

Neuropathology Appl Neurobio

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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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Section: Discussionsupporting

confidence: 77%