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Genetic heterogeneity of neuronal intranuclear inclusion disease: What about the infantile variant?

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F21%3A10427922" target="_blank" >RIV/00216208:11110/21:10427922 - isvavai.cz</a>

  • Alternative codes found

    RIV/00064165:_____/21:10427922

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=AnfmpDhZCx" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=AnfmpDhZCx</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1002/acn3.51332" target="_blank" >10.1002/acn3.51332</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Genetic heterogeneity of neuronal intranuclear inclusion disease: What about the infantile variant?

  • Original language description

    Chen et al. have demonstrated lack of abnormal CGG expansions in the NOTCH2NLC gene among European juvenile and adult patients with neuronal intranuclear inclusion disease (jNIID and aNIID). Genetic heterogeneity of NIID was suggested based on the discrepancy of these results and earlier findings in East Asian patients. We recently studied NOTCH2NLC CGG repeats in an infantile NIID (iNIID) patient independently of Chen et al. This Caucasian European boy is the first, and thus far only, iNIID patient who has been tested for this molecular pathology. The number of repeats in this patient corresponded to values found in healthy controls. We believe that these data provide further evidence for genetic heterogeneity of NIID. It is, however, important to highlight that our results do not, at this point, allow conclusions about genetic causes of NIID among European patients of all age groups or about genetic causes of iNIID.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30109 - Pathology

Result continuities

  • Project

    <a href="/en/project/NV19-08-00137" target="_blank" >NV19-08-00137: The application of new methods of genomic analysis in cases of rare genetic based diseases with negative results of genetic and genomic analyses.</a><br>

  • Continuities

    P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Others

  • Publication year

    2021

  • Confidentiality

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

Data specific for result type

  • Name of the periodical

    Annals of Clinical and Translational Neurology [online]

  • ISSN

    2328-9503

  • e-ISSN

  • Volume of the periodical

    8

  • Issue of the periodical within the volume

    4

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    8

  • Pages from-to

    994-1001

  • UT code for WoS article

    000634321700001

  • EID of the result in the Scopus database

    2-s2.0-85103225628