Abstract:
:Gaucher disease (GD) is a lysosomal storage disorder, caused by an impaired function of β-glucocerebrosidase, which results in accumulation of glucocerebroside in cells, and altered membrane ordering. Using electron paramagnetic resonance spin labeling, a statistically significant difference in the order parameter between the peripheral blood mononuclear cell membranes of GD patients and healthy controls was observed. Moreover, the results show that the introduction of the enzyme replacement therapy leads to the restoration of the physiological membrane fluidity. Accordingly, this simple method could serve as a preliminary test for GD diagnosis and therapy efficiency.
journal_name
Biol Chemjournal_title
Biological chemistryauthors
Pavićević A,Lakočević M,Popović M,Popović-Bijelić A,Daković M,Mojović Mdoi
10.1515/hsz-2017-0241subject
Has Abstractpub_date
2018-04-25 00:00:00pages
447-452issue
5eissn
1431-6730issn
1437-4315pii
/j/bchm.just-accepted/hsz-2017-0241/hsz-2017-0241.journal_volume
399pub_type
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