Journal ArticleDOI
Functional and morphometric study of the liver in motor neuron disease.
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TLDR
The pathogenetic processes in MND may involve not only motor neurons but also hepatic cells, and the MND patients had a higher incidence of intramitochondrial inclusions, less abundant mitochondria in a given area of cytoplasm and enlarged mitochondria.Abstract:
In routine liver function tests, 23 of 44 patients with motor neuron disease (MND) had abnormal findings, and there was disturbance of unconjugated bilirubin metabolism in 10 of the 33 patients tested. Liver-biopsy specimens from 10 MND patients were compared by electron microscopic examination with specimens from age-matched controls who had chronic persistent hepatitis. The MND patients had a higher incidence of intramitochondrial inclusions, less abundant mitochondria in a given area of cytoplasm and enlarged mitochondria. Electron-probe X-ray microanalysis of hepatocytic lysosomes found copper in 8 of 13 MND patients, but not in the controls. These findings suggest that the pathogenetic processes in MND may involve not only motor neurons but also hepatic cells.read more
Citations
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TL;DR: A concise overview of the major findings in recent years highlighting the importance of healthy mitochondria for a healthy neuron in neurodegenerative diseases is provided.
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TL;DR: Mitochondrial electron trans port, Ca2+ transport, free radical production, and partici pation in the apoptotic cascade were addressed in a companion review in the March 1999 issue of the Lancet.
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Mitochondrial dysfunction and oxidative stress in aging and neurodegenerative disease.
David S. Albers,M. Flint Beal +1 more
TL;DR: The age-dependent onset and progressive course of these neurodegenerative diseases may ultimately highlight an association between aging, mitochondrial impairment and oxidative stress.
References
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Journal ArticleDOI
Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis
TL;DR: It is shown that in amyotrophic lateral sclerosis a small degree of dying‐back change and of distal axonal atrophy is superimposed on the degeneration of motor neuron cell bodies, and that the disease effects spread beyond the motor neurons.
Journal Article
Changes in the distribution of hepatic copper in relation to the progression of Wilson's disease (hepatolenticular degeneration).
S Goldfischer,I Sternlieb +1 more
Journal Article
The significance of variations in the distribution of copper in liver disease.
TL;DR: Differences in the distribution of copper and the cellular changes associated with its deposition suggest that different pathogenetic mechanisms and possibly different intracellular targets are susceptible to the toxic effects of the metal.