Changes of the peripheral blood mononuclear cells membrane fluidity from type 1 Gaucher disease patients: an electron paramagnetic resonance study.

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 Chem

journal_title

Biological chemistry

authors

Pavićević A,Lakočević M,Popović M,Popović-Bijelić A,Daković M,Mojović M

doi

10.1515/hsz-2017-0241

subject

Has Abstract

pub_date

2018-04-25 00:00:00

pages

447-452

issue

5

eissn

1431-6730

issn

1437-4315

pii

/j/bchm.just-accepted/hsz-2017-0241/hsz-2017-0241.

journal_volume

399

pub_type

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