Number of the records: 1
Ultrastructural and functional abnormalities of mitochondria in cultivated fibroblasts from α -mannosidosis patiens
- 1.
SYSNO ASEP 0338472 Document Type J - Journal Article R&D Document Type Journal Article Subsidiary J Článek ve WOS Title Ultrastructural and functional abnormalities of mitochondria in cultivated fibroblasts from α -mannosidosis patiens Title Abnormality v ultrastruktuře a funkci mitochondrií ve fibroblastech kultivovaných od pacientů s alfa mannosidosou Author(s) Brantová, O. (CZ)
Asfaw, B. (CZ)
Sládková, J. (CZ)
Poupětová, H. (CZ)
Živný, J. (CZ)
Magner, M. (CZ)
Krůšek, Jan (FGU-C) RID, ORCID
Veselá, K. (CZ)
Hansíková, H. (CZ)
Ledvinová, J. (CZ)
Tesařová, M. (CZ)
Zeman, J. (CZ)Source Title Biologia. - : Springer - ISSN 0006-3088
Roč. 64, č. 2 (2009), s. 394-401Number of pages 8 s. Language eng - English Country SK - Slovakia Keywords mitochondria ; endoplasmatic reticulum ; ultrastructure Subject RIV ED - Physiology CEZ AV0Z50110509 - FGU-C (2005-2011) UT WOS 000263540800027 DOI 10.2478/s11756-009-0054-2 Annotation Alfa-Mannosidosis is a lysosomal storage disorder caused by alfa-mannosidase defficiency. The aim of the study was to analyse mitochondrial ultrastructure and function in cultivated fibroblasts from three pacients with alfa-mannosidase. In analyzed fibroblasts we observed dilated ER, higher amount of aberrant mitochondria and reduced mitochondrial mass compared to controls Workplace Institute of Physiology Contact Lucie Trajhanová, lucie.trajhanova@fgu.cas.cz, Tel.: 241 062 400 Year of Publishing 2010
Number of the records: 1