Genetic architecture of childhood speech disorder: a review.


Journal

Molecular psychiatry
ISSN: 1476-5578
Titre abrégé: Mol Psychiatry
Pays: England
ID NLM: 9607835

Informations de publication

Date de publication:
16 Feb 2024
Historique:
received: 02 08 2023
accepted: 02 01 2024
revised: 20 12 2023
medline: 17 2 2024
pubmed: 17 2 2024
entrez: 17 2 2024
Statut: aheadofprint

Résumé

Severe speech disorders lead to poor literacy, reduced academic attainment and negative psychosocial outcomes. As early as the 1950s, the familial nature of speech disorders was recognized, implying a genetic basis; but the molecular genetic basis remained unknown. In 2001, investigation of a large three generational family with severe speech disorder, known as childhood apraxia of speech (CAS), revealed the first causative gene; FOXP2. A long hiatus then followed for CAS candidate genes, but in the past three years, genetic analysis of cohorts ascertained for CAS have revealed over 30 causative genes. A total of 36 pathogenic variants have been identified from 122 cases across 3 cohorts in this nascent field. All genes identified have been in coding regions to date, with no apparent benefit at this stage for WGS over WES in identifying monogenic conditions associated with CAS. Hence current findings suggest a remarkable one in three children have a genetic variant that explains their CAS, with significant genetic heterogeneity emerging. Around half of the candidate genes identified are currently supported by medium (6 genes) to strong (9 genes) evidence supporting the association between the gene and CAS. Despite genetic heterogeneity; many implicated proteins functionally converge on pathways involved in chromatin modification or transcriptional regulation, opening the door to precision diagnosis and therapies. Most of the new candidate genes for CAS are associated with previously described neurodevelopmental conditions that include intellectual disability, autism and epilepsy; broadening the phenotypic spectrum to a distinctly milder presentation defined by primary speech disorder in the setting of normal intellect. Insights into the genetic bases of CAS, a severe, rare speech disorder, are yet to translate to understanding the heritability of more common, typically milder forms of speech or language impairment such as stuttering or phonological disorder. These disorders likely follow complex inheritance with polygenic contributions in many cases, rather than the monogenic patterns that underly one-third of patients with CAS. Clinical genetic testing for should now be implemented for individuals with CAS, given its high diagnostic rate, which parallels many other neurodevelopmental disorders where this testing is already standard of care. The shared mechanisms implicated by gene discovery for CAS highlight potential new targets for future precision therapies.

Identifiants

pubmed: 38366112
doi: 10.1038/s41380-024-02409-8
pii: 10.1038/s41380-024-02409-8
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Department of Health | National Health and Medical Research Council (NHMRC)
ID : 1105008
Organisme : Department of Health | National Health and Medical Research Council (NHMRC)
ID : 1195955
Organisme : Department of Health | National Health and Medical Research Council (NHMRC)
ID : 1153614

Informations de copyright

© 2024. The Author(s).

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Auteurs

Angela T Morgan (AT)

Murdoch Children's Research Institute, Melbourne, VIC, Australia. angela.morgan@mcri.edu.au.
Speech Pathology, University of Melbourne, Melbourne, VIC, Australia. angela.morgan@mcri.edu.au.
Speech Pathology, Royal Children's Hospital, Melbourne, VIC, Australia. angela.morgan@mcri.edu.au.

David J Amor (DJ)

Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Department of Paediatrics, University of Melbourne, Royal Children's Hospital, Melbourne, VIC, Australia.

Miya D St John (MD)

Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Speech Pathology, University of Melbourne, Melbourne, VIC, Australia.

Ingrid E Scheffer (IE)

Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Epilepsy Research Centre, Austin Health, Melbourne, VIC, Australia.

Michael S Hildebrand (MS)

Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Epilepsy Research Centre, Austin Health, Melbourne, VIC, Australia.

Classifications MeSH