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Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability

Intellectual disability (ID) is a condition of significant limitation of cognitive functioning and adaptive behavior, with 50% of etiology attributed to genetic predisposition. We recruited two consanguineous Pakistani families manifesting severe ID and developmental delay. The probands were subject...

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Autores principales: Asif, Maria, Anayat, Maryam, Tariq, Faiza, Noureen, Tanzeela, Din, Ghulam Naseer Ud, Becker, Christian, Becker, Kerstin, Thiele, Holger, Makhdoom, Ehtisham ul Haq, Shaiq, Pakeeza Arzoo, Baig, Shahid M., Nürnberg, Peter, Hussain, Muhammad Sajid, Raja, Ghazala Kaukab, Abdullah, Uzma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9858807/
https://www.ncbi.nlm.nih.gov/pubmed/36672789
http://dx.doi.org/10.3390/genes14010048
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author Asif, Maria
Anayat, Maryam
Tariq, Faiza
Noureen, Tanzeela
Din, Ghulam Naseer Ud
Becker, Christian
Becker, Kerstin
Thiele, Holger
Makhdoom, Ehtisham ul Haq
Shaiq, Pakeeza Arzoo
Baig, Shahid M.
Nürnberg, Peter
Hussain, Muhammad Sajid
Raja, Ghazala Kaukab
Abdullah, Uzma
author_facet Asif, Maria
Anayat, Maryam
Tariq, Faiza
Noureen, Tanzeela
Din, Ghulam Naseer Ud
Becker, Christian
Becker, Kerstin
Thiele, Holger
Makhdoom, Ehtisham ul Haq
Shaiq, Pakeeza Arzoo
Baig, Shahid M.
Nürnberg, Peter
Hussain, Muhammad Sajid
Raja, Ghazala Kaukab
Abdullah, Uzma
author_sort Asif, Maria
collection PubMed
description Intellectual disability (ID) is a condition of significant limitation of cognitive functioning and adaptive behavior, with 50% of etiology attributed to genetic predisposition. We recruited two consanguineous Pakistani families manifesting severe ID and developmental delay. The probands were subjected to whole exome sequencing (WES) and variants were further prioritized based on population frequency, predicted pathogenicity and functional relevance. The WES data analysis identified homozygous pathogenic variants in genes MBOAT7 and TRAPPC9. The pathogenicity of the variants was supported by co-segregation analysis and in silico tool. The findings of this study expand mutation spectrum and provide additional evidence to the role of MBOAT7 and TRAPPC9 in causation of ID.
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spelling pubmed-98588072023-01-21 Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability Asif, Maria Anayat, Maryam Tariq, Faiza Noureen, Tanzeela Din, Ghulam Naseer Ud Becker, Christian Becker, Kerstin Thiele, Holger Makhdoom, Ehtisham ul Haq Shaiq, Pakeeza Arzoo Baig, Shahid M. Nürnberg, Peter Hussain, Muhammad Sajid Raja, Ghazala Kaukab Abdullah, Uzma Genes (Basel) Article Intellectual disability (ID) is a condition of significant limitation of cognitive functioning and adaptive behavior, with 50% of etiology attributed to genetic predisposition. We recruited two consanguineous Pakistani families manifesting severe ID and developmental delay. The probands were subjected to whole exome sequencing (WES) and variants were further prioritized based on population frequency, predicted pathogenicity and functional relevance. The WES data analysis identified homozygous pathogenic variants in genes MBOAT7 and TRAPPC9. The pathogenicity of the variants was supported by co-segregation analysis and in silico tool. The findings of this study expand mutation spectrum and provide additional evidence to the role of MBOAT7 and TRAPPC9 in causation of ID. MDPI 2022-12-23 /pmc/articles/PMC9858807/ /pubmed/36672789 http://dx.doi.org/10.3390/genes14010048 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Asif, Maria
Anayat, Maryam
Tariq, Faiza
Noureen, Tanzeela
Din, Ghulam Naseer Ud
Becker, Christian
Becker, Kerstin
Thiele, Holger
Makhdoom, Ehtisham ul Haq
Shaiq, Pakeeza Arzoo
Baig, Shahid M.
Nürnberg, Peter
Hussain, Muhammad Sajid
Raja, Ghazala Kaukab
Abdullah, Uzma
Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability
title Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability
title_full Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability
title_fullStr Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability
title_full_unstemmed Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability
title_short Whole-Exome Sequencing of Pakistani Consanguineous Families Identified Pathogenic Variants in Genes of Intellectual Disability
title_sort whole-exome sequencing of pakistani consanguineous families identified pathogenic variants in genes of intellectual disability
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9858807/
https://www.ncbi.nlm.nih.gov/pubmed/36672789
http://dx.doi.org/10.3390/genes14010048
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