Shared Biological Pathways and Processes in Patients with Intellectual Disability: A Multicenter Study.


Journal

Neuropediatrics
ISSN: 1439-1899
Titre abrégé: Neuropediatrics
Pays: Germany
ID NLM: 8101187

Informations de publication

Date de publication:
08 2023
Historique:
medline: 12 7 2023
pubmed: 15 2 2023
entrez: 14 2 2023
Statut: ppublish

Résumé

Although the underlying genetic causes of intellectual disability (ID) continue to be rapidly identified, the biological pathways and processes that could be targets for a potential molecular therapy are not yet known. This study aimed to identify ID-related shared pathways and processes utilizing enrichment analyses. In this multicenter study, causative genes of patients with ID were used as input for Disease Ontology (DO), Gene Ontology (GO), and Kyoto Encyclopedia of Genes and Genomes enrichment analysis. Genetic test results of 720 patients from 27 centers were obtained. Patients with chromosomal deletion/duplication, non-ID genes, novel genes, and results with changes in more than one gene were excluded. A total of 558 patients with 341 different causative genes were included in the study. Pathway-based enrichment analysis of the ID-related genes via ClusterProfiler revealed 18 shared pathways, with lysine degradation and nicotine addiction being the most common. The most common of the 25 overrepresented DO terms was ID. The most frequently overrepresented GO biological process, cellular component, and molecular function terms were regulation of membrane potential, ion channel complex, and voltage-gated ion channel activity/voltage-gated channel activity, respectively. Lysine degradation, nicotine addiction, and thyroid hormone signaling pathways are well-suited to be research areas for the discovery of new targeted therapies in ID patients.

Sections du résumé

BACKGROUND
Although the underlying genetic causes of intellectual disability (ID) continue to be rapidly identified, the biological pathways and processes that could be targets for a potential molecular therapy are not yet known. This study aimed to identify ID-related shared pathways and processes utilizing enrichment analyses.
METHODS
In this multicenter study, causative genes of patients with ID were used as input for Disease Ontology (DO), Gene Ontology (GO), and Kyoto Encyclopedia of Genes and Genomes enrichment analysis.
RESULTS
Genetic test results of 720 patients from 27 centers were obtained. Patients with chromosomal deletion/duplication, non-ID genes, novel genes, and results with changes in more than one gene were excluded. A total of 558 patients with 341 different causative genes were included in the study. Pathway-based enrichment analysis of the ID-related genes via ClusterProfiler revealed 18 shared pathways, with lysine degradation and nicotine addiction being the most common. The most common of the 25 overrepresented DO terms was ID. The most frequently overrepresented GO biological process, cellular component, and molecular function terms were regulation of membrane potential, ion channel complex, and voltage-gated ion channel activity/voltage-gated channel activity, respectively.
CONCLUSION
Lysine degradation, nicotine addiction, and thyroid hormone signaling pathways are well-suited to be research areas for the discovery of new targeted therapies in ID patients.

Identifiants

pubmed: 36787800
doi: 10.1055/a-2034-8528
doi:

Substances chimiques

Lysine K3Z4F929H6
Ion Channels 0

Types de publication

Multicenter Study Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

225-238

Informations de copyright

Thieme. All rights reserved.

Déclaration de conflit d'intérêts

None declared.

Auteurs

Çağatay Günay (Ç)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Duygu Aykol (D)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Özlem Özsoy (Ö)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Ece Sönmezler (E)

Izmir Biomedicine and Genome Center, Dokuz Eylul University Health Campus, Izmir, Turkey.

Yaren Sena Hanci (YS)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Bülent Kara (B)

Department of Pediatric Neurology, Kocaeli University School of Medicine, Kocaeli, Turkey.

Deniz Akkoyunlu Sünnetçi (D)

Department of Pediatric Neurology, Kocaeli University School of Medicine, Kocaeli, Turkey.

Naci Cine (N)

Department of Medical Genetics, Kocaeli University School of Medicine, Kocaeli, Turkey.

Adnan Deniz (A)

Department of Pediatric Neurology, Kocaeli University School of Medicine, Kocaeli, Turkey.

Tolgahan Özer (T)

Department of Medical Genetics, Kocaeli University School of Medicine, Kocaeli, Turkey.

Cemile Büşra Ölçülü (CB)

Department of Child Neurology, Ege University Faculty of Medicine, Izmir, Turkey.

Özlem Yilmaz (Ö)

Department of Child Neurology, Ege University Faculty of Medicine, Izmir, Turkey.

Seda Kanmaz (S)

Department of Child Neurology, Ege University Faculty of Medicine, Izmir, Turkey.

Sanem Yilmaz (S)

Department of Child Neurology, Ege University Faculty of Medicine, Izmir, Turkey.

Hasan Tekgül (H)

Department of Child Neurology, Ege University Faculty of Medicine, Izmir, Turkey.

Nihal Yildiz (N)

Department of Pediatric Neurology, Karadeniz Technical University, Faculty of Medicine, Farabi Hospital, Trabzon, Turkey.

Elif Acar Arslan (E)

Department of Pediatric Neurology, Karadeniz Technical University, Faculty of Medicine, Farabi Hospital, Trabzon, Turkey.

Ali Cansu (A)

Department of Pediatric Neurology, Karadeniz Technical University, Faculty of Medicine, Farabi Hospital, Trabzon, Turkey.

Nihal Olgaç Dündar (N)

Department of Pediatric Neurology, İzmir Katip Çelebi University, Izmir, Turkey.

Fatma Kusgoz (F)

Department of Pediatric Neurology, Tepecik Research and Training Hospital, Izmir, Turkey.

Elif Didinmez (E)

Department of Pediatric Neurology, Tepecik Research and Training Hospital, Izmir, Turkey.

Pınar Gençpinar (P)

Department of Pediatric Neurology, İzmir Katip Çelebi University, Izmir, Turkey.

Tuğçe Aksu Uzunhan (T)

Department of Pediatric Neurology, Prof Dr Cemil Tascioglu City Hospital, Istanbul, Turkey.

Biray Ertürk (B)

Department of Pediatric Neurology, Prof Dr Cemil Tascioglu City Hospital, Istanbul, Turkey.

Alper Gezdirici (A)

Department of Medical Genetics, Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey.

Akif Ayaz (A)

Department of Medical Genetics, Istanbul Medipol University School of Medicine, Istanbul, Turkey.

Akgün Ölmez (A)

Denizli Pediatric Neurology Clinic, Denizli, Turkey.

Müge Ayanoğlu (M)

Department of Child Neurology, Adnan Menderes University School of Medicine, Aydın, Turkey.

Ayşe Tosun (A)

Department of Child Neurology, Adnan Menderes University School of Medicine, Aydın, Turkey.

Yasemin Topçu (Y)

Department of Pediatric Neurology, Istanbul Medipol University Faculty of Medicine, Istanbul, Turkey.

Betül Kiliç (B)

Department of Pediatric Neurology, Istanbul Medipol University Faculty of Medicine, Istanbul, Turkey.

Kürşad Aydin (K)

Department of Pediatric Neurology, Istanbul Medipol University Faculty of Medicine, Istanbul, Turkey.

Ezgi Çağlar (E)

Departments of Pediatric Neurology, Mersin University Faculty of Medicine, Mersin, Turkey.

Özlem Ersoy Kosvali (Ö)

Departments of Pediatric Neurology, Mersin University Faculty of Medicine, Mersin, Turkey.

Çetin Okuyaz (Ç)

Departments of Pediatric Neurology, Mersin University Faculty of Medicine, Mersin, Turkey.

Şeyda Besen (Ş)

Division of Pediatric Neurology, Başkent University Adana Medical and Research Center Faculty of Medicine, Adana, Turkey.

Leman Tekin Orgun (L)

Division of Pediatric Neurology, Başkent University Adana Medical and Research Center Faculty of Medicine, Adana, Turkey.

İlknur Erol (İ)

Division of Pediatric Neurology, Başkent University Adana Medical and Research Center Faculty of Medicine, Adana, Turkey.

Deniz Yüksel (D)

Department of Pediatric Neurology, University of Health Sciences Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Abdullah Sezer (A)

Department of Genetics, University of Health Sciences Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Ergin Atasoy (E)

Department of Pediatric Neurology, University of Health Sciences Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Ülkühan Toprak (Ü)

Department of Pediatric Neurology, University of Health Sciences Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Serdal Güngör (S)

Department of Paediatric Neurology, Inonu University Faculty of Medicine, Turgut Ozal Research Center, Malatya, Turkey.

Bilge Ozgor (B)

Department of Paediatric Neurology, Inonu University Faculty of Medicine, Turgut Ozal Research Center, Malatya, Turkey.

Meral Karadağ (M)

Department of Paediatric Neurology, Inonu University Faculty of Medicine, Turgut Ozal Research Center, Malatya, Turkey.

Cengiz Dilber (C)

Department of Pediatric Neurology, Kahramanmaras Sutcu Imam University Faculty of Medicine, Kahramanmaraş, Turkey.

Bahtiyar Şahinoğlu (B)

Deparment of Genetics, Dr Ersin Arslan Traning and Research Hospital, Gaziantep, Turkey.

Emek Uyur Yalçin (E)

Departments of Pediatrics and Pediatric Neurology, University of Health Sciences, Zeynep Kamil Maternity and Children's Diseases Hospital, Istanbul, Turkey.

Nilüfer Eldes Hacifazlioglu (N)

Departments of Pediatrics and Pediatric Neurology, University of Health Sciences, Zeynep Kamil Maternity and Children's Diseases Hospital, Istanbul, Turkey.

Ahmet Yaramiş (A)

Diyarbakır Pediatric Neurology Clinic, Diyarbakır, Turkey.

Pınar Edem (P)

Department of Pediatric Neurology, Bakırcay University, Cigli District Training Hospital, Izmir, Turkey.

Hande Gezici Tekin (H)

Department of Pediatric Neurology, Bakırcay University, Cigli District Training Hospital, Izmir, Turkey.

Ünsal Yilmaz (Ü)

Department of Pediatric Neurology, Dr. Behcet Uz Children's Hospital, Izmir, Turkey.

Aycan Ünalp (A)

Department of Pediatric Neurology, Dr. Behcet Uz Children's Hospital, Izmir, Turkey.

Sevim Turay (S)

Department of Pediatric Neurology, Duzce University Faculty of Medicine, Düzce, Turkey.

Didem Biçer (D)

Department of Pediatric Neurology, Çukurova University Faculty of Medicine, Adana, Turkey.

Gülen Gül Mert (G)

Department of Pediatric Neurology, Çukurova University Faculty of Medicine, Adana, Turkey.

İpek Dokurel Çetin (İ)

Department of Pediatric Neurology, Balıkesir Atatürk Training and Research Hospital, Balıkesir, Turkey.

Serkan Kirik (S)

Fırat University School of Medicine, Pediatric Neurology, Elazığ, Turkey.

Gülten Öztürk (G)

Department of Pediatric Neurology, Marmara University School of Medicine, Istanbul, Turkey.

Yasemin Karal (Y)

Department of Pediatric Neurology, Trakya University, Faculty of Medicine, Edirne, Turkey.

Aslıhan Sanri (A)

Department of Pediatric Genetics, University of Health Sciences, Samsun Training and Research Hospital, Samsun, Turkey.

Ayşe Aksoy (A)

Department of Pediatric Neurology, Ondokuz Mayıs University, Samsun, Turkey.

Muzaffer Polat (M)

Department of Pediatric Neurology, Celal Bayar University School of Medicine, Manisa, Turkey.

Nezir Özgün (N)

Department of Pediatric Neurology, Mardin Artuklu University, Faculty of Health Sciences, Mardin, Turkey.

Didem Soydemir (D)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Gamze Sarikaya Uzan (G)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Döndü Ülker Üstebay (D)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Ayşen Gök (A)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Mehmet Can Yeşilmen (MC)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Uluç Yiş (U)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.

Gökhan Karakülah (G)

Izmir Biomedicine and Genome Center, Dokuz Eylul University Health Campus, Izmir, Turkey.

Ahmet Bursali (A)

Izmir Biomedicine and Genome Center, Dokuz Eylul University Health Campus, Izmir, Turkey.

Yavuz Oktay (Y)

Izmir Biomedicine and Genome Center, Dokuz Eylul University Health Campus, Izmir, Turkey.

Semra Hiz Kurul (S)

Department of Pediatric Neurology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.
Izmir Biomedicine and Genome Center, Dokuz Eylul University Health Campus, Izmir, Turkey.

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