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Analysis of histopathologic and molecular pathologic findings in czech LGMD2A patients

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216224%3A14310%2F06%3A00017994" target="_blank" >RIV/00216224:14310/06:00017994 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/06:93 RIV/00216208:11130/06:3072 RIV/00216224:14110/05:00021640

  • Result on the web

  • DOI - Digital Object Identifier

Alternative languages

  • Result language

    angličtina

  • Original language name

    Analysis of histopathologic and molecular pathologic findings in czech LGMD2A patients

  • Original language description

    Histopathologic and molecular pathologic findings were examined in 14 LGMD2A patients. Analysis of the CAPN3 gene was performed at the mRNA level and/or DNA level. Immunoblot analysis revealed the deficiency of p94 in all genetically confirmed cases, andsecondary dysferlin deficiency was demonstrated in 6 patients. In muscle tissues of two patients, a neurogenic pattern simulating neurogenic lesion was observed.

  • Czech name

    Analýza histopatologických a molekulárně patologických nálezů u českých pacientů s LGMD2A

  • Czech description

    U 14 pacientů s diagnózou LGMD2A byly analyzovány histopatologické a molekulárně genetické nálezy. Analýza genu CAPN3 byla provedena na úrovni mRNA a/nebo DNA. Analýza proteinů Western blotem odhalila deficit kalpainu-3 u všech pacientů s geneticky potvrzenou diagnózou LGMD2A. Sekundární deficit dysferlinu byl detekován u 6 pacientů. Ve svalové tkáni dvou pacientů byla pozorována nerogenní léze.

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    EB - Genetics and molecular biology

  • OECD FORD branch

Result continuities

  • Project

    <a href="/en/project/NR8087" target="_blank" >NR8087: Diagnostics of neurodegenerative muscular disorders using mRNA/DNA analysis of selected genes and chromosome loci</a><br>

  • Continuities

    Z - Vyzkumny zamer (s odkazem do CEZ)

Others

  • Publication year

    2006

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Muscle & Nerve

  • ISSN

    0148-639X

  • e-ISSN

  • Volume of the periodical

    33

  • Issue of the periodical within the volume

    March

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    9

  • Pages from-to

    424

  • UT code for WoS article

  • EID of the result in the Scopus database