Title:

WPŁYW SUROWIC CHORYCH ZE ZWYRODNIENIEM WĄTROBOWO-SOCZEWKOWYM LECZONYCH D-PENICYLAMINĄ NA ROZWÓJ GLIOPATII WILSONOWSKIEJ 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:

Czasopismo/Artykuł

Subject and Keywords:

Zwyrodnienie wątrobowo-soczewkowe. ; D-penicylamina ; Gliopatia Wilsonowska

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:

Tekst

Detailed Resource Type:

Artykuł

Format:

pdf

Language:

pol

Language of abstract:

eng ; rus

Rights:

Licencja Creative Commons Uznanie autorstwa 4.0

Terms of use:

Zasób chroniony prawem autorskim. [CC BY 4.0 Międzynarodowe] Korzystanie dozwolone zgodnie z licencją Creative Commons Uznanie autorstwa 4.0, której pełne postanowienia dostępne są pod adresem: ; -

Digitizing institution:

Instytut Medycyny Doświadczalnej i Klinicznej im. M. Mossakowskiego Polskiej Akademii Nauk

Original in:

Biblioteka Instytutu Medycyny Doświadczalnej i Klinicznej im. M. Mossakowskiego PAN

Projects co-financed by:

Program Operacyjny Polska Cyfrowa, lata 2014-2020, Działanie 2.3 : Cyfrowa dostępność i użyteczność sektora publicznego; środki z Europejskiego Funduszu Rozwoju Regionalnego oraz współfinansowania krajowego z budżetu państwa

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