Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyotrophic lateral sclerosis (ALS) is a devastating, neurodegenerative motor neuron disease. The aetiology of ALS remains an enigma which hinders the design of an effective treatment to prevent, postpone, or reverse the pathophysiological changes occurring during the aggressive progression of this disease. During the last decade, basic research within the innate immune system, and in particular the complement system, has revealed new, important roles of the innate immune system during development, homeostasis, and ageing within as well as outside the central nervous system. Several lines of evidence indicate that aberrant activation of the complement system locally in the central nervous system as well as systemically may be involved in the pathophysiology of ALS. This exciting new knowledge could point towards the innate immune system as a potential target of medical intervention in ALS. Recently, the historic perception of ALS as a central neurodegenerative disease has been challenged due to the significant amount of evidence of a dying-back mechanism causing the selective destruction of the motor neurons, indicating that disease onset occurs outside the borders of the blood-brain-barrier. This review addresses the function of the innate immune system during ALS. We emphasize the role of the complement system and specifically suggest the involvement of ficolin-3 from the lectin pathway in the pathophysiology of ALS.
...
PMID:Amyotrophic lateral sclerosis: The complement and inflammatory hypothesis. 2993 90

In amyotrophic lateral sclerosis, there is a need for biomarkers to distinguish patients from controls, to follow disease progression and to provide information about the pathogenesis of disease. In a previous mass spectrometry study that searched for potential proteins of interest, we identified clusterin, CD5L, ficolin-3, and gelsolin as molecules that differed in abundance between ALS patients and controls, with a greater difference in patients with cognitive impairment. Here, we have measured levels of these molecules in plasma from a separate cohort of ALS patients and controls. The plasma was depleted of abundant plasma proteins. We confirmed our previous findings that levels of clusterin are decreased and ficolin-3 are increased in ALS patients compared to controls. In this study, we found that levels of CD5L were increased in patients with ALS and levels correlated with survival. We found that levels of gelsolin were modestly increased in ALS compared to controls whereas in our previous study these were decreased, especially in patients with cognitive impairment who were not included in this study. We suggest that clusterin and ficolin-3 deserve further study as potential ALS biomarkers.
...
PMID:Levels of clusterin, CD5L, ficolin-3, and gelsolin in ALS patients and controls. 3255 99