Title:

Effect of sera from patients with hepatolenticular degeneration treated with D-penicillamine on the development of Wilsonian gliopathy in vitro

Creator:

Weinrauder, Halina ; Mossakowski, Mirosław Jan (1929–2001) ; Ganuszkina, IV.

Publisher:

Zakład im. Ossolińskich - Wydawnictwo Polskiej Akademii Nauk

Place of publishing:

Wrocław ; Warszawa ; Kraków

Date issued/created:

1986

Type of object:

Journal/Article

Subject and Keywords:

hepatolenticular degeneration ; D-penicillamine ; Wilsonian gliopathy

Abstract:

Studies on the influence of sera from patients with Wilson’s disease treated with d-penicilamine upon morphology and immunomorphology of astrocytes in organotypic cultures of newborn rat cerebella were performed. The pathological sera applied were characterized by significant reduction of both copper level and céruloplasmine activity. Remarkable differences were found as compared with the influence of sera from untreated patients. They consisted in greater cellula-rity of cultures and predominance of astrocytic hypertrophy over nonspecific cel¬lular degeneration. However, astrocytic abnormalities, typical for Wilsonian gliopathy such as Alzheimer cells, type II and Opalski cells were also present. Intensity of degenerative changes of astrocytes and frequency of Alzheimer cells type II and Opalski cells appearance were correlated with the advancement of the disease process and degree of céruloplasmine activity reduction. All ab¬normal astrocytes both showing features of hypertrophy and degeneration gave positive immunofluorescent reaction with anti-GFAP serum. So did Alzheimer cells, type II and Opalski cells. The authors supposed that less severe astrocytic abnormalities as compared with those obtained with sera from untreated patients were due to lowered copper content in sera used in present experiments. However,disturbed pro¬portion between bound and free copper in sera resulting from reduction of céru¬loplasmine activity could be responsible for the appearance of Wilsonian gliopathy features, even unde rconditions of lowered general content of copper.

Relation:

Neuropatologia Polska

Volume:

24

Issue:

2

Start page:

169

End page:

181

Resource type:

Text

Detailed Resource Type:

Article

Format:

pdf

Language:

pol

Language of abstract:

eng ; rus

Rights:

Creative Commons Attribution BY 4.0 license

Terms of use:

Copyright-protected material. [CC BY 4.0] May be used within the scope specified in Creative Commons Attribution BY 4.0 license, full text available at: ; -

Digitizing institution:

Mossakowski Medical Research Institute PAS

Original in:

Library of the Mossakowski Medical Research Institute PAS

Projects co-financed by:

Operational Program Digital Poland, 2014-2020, Measure 2.3: Digital accessibility and usefulness of public sector information; funds from the European Regional Development Fund and national co-financing from the state budget.

Access:

Open