Glucocerebrosidase inhibition causes mitochondrial dysfunction and free radical damage
نویسندگان
چکیده
منابع مشابه
Glucocerebrosidase inhibition causes mitochondrial dysfunction and free radical damage
Mutations of the gene for glucocerebrosidase 1 (GBA) cause Gaucher disease (GD), an autosomal recessive lysosomal storage disorder. Individuals with homozygous or heterozygous (carrier) mutations of GBA have a significantly increased risk for the development of Parkinson's disease (PD), with clinical and pathological features that mirror the sporadic disease. The mechanisms whereby GBA mutation...
متن کاملFree Radical Damage and Antioxidant Intervention Free radicals and mitochondrial dysfunction in Parkinson’s disease
Introduction The pathological hallmark of Parkinson’s disease (PD) is the death of dopaminergic neurons in the substantia nigra. This results in the characteristic clinical triad of bradykinesia, rigidity and tremor, all of which may be relieved by treatment with ~-3 ,4dihydroxyphenylalanine (DOPA). Treatment is only symptomatic, however, and its efficacy wanes as dopamine-containing neurons co...
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متن کاملMitochondrial dysfunction associated with glucocerebrosidase deficiency
The lysosomal hydrolase glucocerebrosidase (GCase) is encoded for by the GBA gene. Homozygous GBA mutations cause Gaucher disease (GD), a lysosomal storage disorder. Furthermore, homozygous and heterozygous GBA mutations are numerically the greatest genetic risk factor for developing Parkinson's disease (PD), the second most common neurodegenerative disorder. The loss of GCase activity results ...
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Background: Three types of cell lines have been established from mouse blastocysts: embryonic stem (ES) cells, trophoblast stem (TS) cells, and extra-embryonic endoderm (XEN) cells, which have the potential to differentiate into their respective cognate lineages. ES cells can differentiate in vitro not only into somatic cell lineages but into extra-embryonic lineages, including trophectoderm an...
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ژورنال
عنوان ژورنال: Neurochemistry International
سال: 2013
ISSN: 0197-0186
DOI: 10.1016/j.neuint.2012.10.010