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Early embryogenesis in CHDFIDD mouse model reveals facial clefts and altered cranial neurogenesis

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985904%3A_____%2F24%3A00587905" target="_blank" >RIV/67985904:_____/24:00587905 - isvavai.cz</a>

  • Alternative codes found

    RIV/68378050:_____/24:00587905 RIV/00216224:14310/24:00136267

  • Result on the web

    <a href="https://journals.biologists.com/dmm/article/17/6/dmm050261/358081/Early-embryogenesis-in-CHDFIDD-mouse-model-reveals" target="_blank" >https://journals.biologists.com/dmm/article/17/6/dmm050261/358081/Early-embryogenesis-in-CHDFIDD-mouse-model-reveals</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1242/dmm.050261" target="_blank" >10.1242/dmm.050261</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Early embryogenesis in CHDFIDD mouse model reveals facial clefts and altered cranial neurogenesis

  • Original language description

    CDK13-related disorder, also known as congenital heart defects, dysmorphic facial features and intellectual developmental disorder (CHDFIDD) is associated with mutations in the CDK13 gene encoding transcription-regulating cyclin-dependent kinase 13 (CDK13). Here, we focused on the development of craniofacial structures and analyzed early embryonic stages in CHDFIDD mouse models, with one model comprising a hypomorphic mutation in Cdk13 and exhibiting cleft lip/palate, and another model comprising knockout of Cdk13 , featuring a stronger phenotype including midfacial cleft. Cdk13 was found to be physiologically expressed at high levels in the mouse embryonic craniofacial structures, namely in the forebrain, nasal epithelium and maxillary mesenchyme. We also uncovered that Cdk13 deficiency leads to development of hypoplastic branches of the trigeminal nerve including the maxillary branch. Additionally, we detected significant changes in the expression levels of genes involved in neurogenesis ( Ache , Dcx , Mef2c , Neurog1 , Ntn1, Pou4f1 ) within the developing palatal shelves. These results, together with changes in the expression pattern of other key facespecific genes ( Fgf8, Foxd1 , Msx1 , Meis2 and Shh ) at early stages in Cdk13 mutant embryos, demonstrate a key role of CDK13 in the regulation of craniofacial morphogenesis.

  • 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

    10601 - Cell biology

Result continuities

  • Project

    Result was created during the realization of more than one project. More information in the Projects tab.

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2024

  • Confidentiality

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

Data specific for result type

  • Name of the periodical

    Disease Models & Mechanisms

  • ISSN

    1754-8403

  • e-ISSN

    1754-8411

  • Volume of the periodical

    17

  • Issue of the periodical within the volume

    6

  • Country of publishing house

    GB - UNITED KINGDOM

  • Number of pages

    16

  • Pages from-to

    dmm050261

  • UT code for WoS article

    001267747400003

  • EID of the result in the Scopus database

    2-s2.0-85197352924