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2015 ; 125
(5
): 1767-79
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Emerging mechanisms of molecular pathology in ALS
#MMPMID25932674
Peters OM
; Ghasemi M
; Brown RH Jr
J Clin Invest
2015[May]; 125
(5
): 1767-79
PMID25932674
show ga
Amyotrophic lateral sclerosis (ALS) is a devastating degenerative disease
characterized by progressive loss of motor neurons in the motor cortex,
brainstem, and spinal cord. Although defined as a motor disorder, ALS can arise
concurrently with frontotemporal lobal dementia (FTLD). ALS begins focally but
disseminates to cause paralysis and death. About 10% of ALS cases are caused by
gene mutations, and more than 40 ALS-associated genes have been identified. While
important questions about the biology of this disease remain unanswered,
investigations of ALS genes have delineated pathogenic roles for (a)
perturbations in protein stability and degradation, (b) altered homeostasis of
critical RNA- and DNA-binding proteins, (c) impaired cytoskeleton function, and
(d) non-neuronal cells as modifiers of the ALS phenotype. The rapidity of
progress in ALS genetics and the subsequent acquisition of insights into the
molecular biology of these genes provide grounds for optimism that meaningful
therapies for ALS are attainable.
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