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Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report
Congenital muscular dystrophies (CMD) are a heterogeneous group of disorders caused by mutations in musculoskeletal proteins. The most common type of CMD in Europe is Merosin-deficient CMD caused by mutations in laminin-α2 protein. Very few studies reported pathogenic variants underlying these disor...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Pacini Editore srl
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598405/ https://www.ncbi.nlm.nih.gov/pubmed/31309178 |
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author | AMIN, MUTAZ BAKHIT, YOUSUF KOKO, MAHMOUD IBRAHIM, MOHAMED OSAMA MIRGAHNI SALIH, MA IBRAHIM, MUNTASER SEIDI, OSHEIK A |
author_facet | AMIN, MUTAZ BAKHIT, YOUSUF KOKO, MAHMOUD IBRAHIM, MOHAMED OSAMA MIRGAHNI SALIH, MA IBRAHIM, MUNTASER SEIDI, OSHEIK A |
author_sort | AMIN, MUTAZ |
collection | PubMed |
description | Congenital muscular dystrophies (CMD) are a heterogeneous group of disorders caused by mutations in musculoskeletal proteins. The most common type of CMD in Europe is Merosin-deficient CMD caused by mutations in laminin-α2 protein. Very few studies reported pathogenic variants underlying these disorders especially from Africa. In this study we report a rare variant (p.Arg148Trp, rs752485547) in LAMA2 gene causing a mild form of Merosin-deficient CMD in a Sudanese family. The family consisted of two patients diagnosed clinically with congenital muscular dystrophy since childhood and five healthy siblings born to consanguineous parents. Whole exome sequencing was performed for the two patients and a healthy sibling. A rare missense variant (p.Arg148Trp, rs752485547) in LAMA2 gene was discovered and verified using Sanger sequencing. The segregation pattern was consistent with autosomal recessive inheritance. The pathogenicity of this variant was predicted using bioinformatics tools. More studies are needed to explore the whole spectrum of mutations in CMD in patients from Sudan and other parts of the world. |
format | Online Article Text |
id | pubmed-6598405 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Pacini Editore srl |
record_format | MEDLINE/PubMed |
spelling | pubmed-65984052019-07-15 Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report AMIN, MUTAZ BAKHIT, YOUSUF KOKO, MAHMOUD IBRAHIM, MOHAMED OSAMA MIRGAHNI SALIH, MA IBRAHIM, MUNTASER SEIDI, OSHEIK A Acta Myol Case Reports Congenital muscular dystrophies (CMD) are a heterogeneous group of disorders caused by mutations in musculoskeletal proteins. The most common type of CMD in Europe is Merosin-deficient CMD caused by mutations in laminin-α2 protein. Very few studies reported pathogenic variants underlying these disorders especially from Africa. In this study we report a rare variant (p.Arg148Trp, rs752485547) in LAMA2 gene causing a mild form of Merosin-deficient CMD in a Sudanese family. The family consisted of two patients diagnosed clinically with congenital muscular dystrophy since childhood and five healthy siblings born to consanguineous parents. Whole exome sequencing was performed for the two patients and a healthy sibling. A rare missense variant (p.Arg148Trp, rs752485547) in LAMA2 gene was discovered and verified using Sanger sequencing. The segregation pattern was consistent with autosomal recessive inheritance. The pathogenicity of this variant was predicted using bioinformatics tools. More studies are needed to explore the whole spectrum of mutations in CMD in patients from Sudan and other parts of the world. Pacini Editore srl 2019-03-01 /pmc/articles/PMC6598405/ /pubmed/31309178 Text en ©2019 Gaetano Conte Academy - Mediterranean Society of Myology, Naples, Italy http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), which permits for noncommercial use, distribution, and reproduction in any digital medium, provided the original work is properly cited and is not altered in any way. For details, please refer to https://creativecommons.org/licenses/by-nc-nd/4.0/ |
spellingShingle | Case Reports AMIN, MUTAZ BAKHIT, YOUSUF KOKO, MAHMOUD IBRAHIM, MOHAMED OSAMA MIRGAHNI SALIH, MA IBRAHIM, MUNTASER SEIDI, OSHEIK A Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report |
title | Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report |
title_full | Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report |
title_fullStr | Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report |
title_full_unstemmed | Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report |
title_short | Rare variant in LAMA2 gene causing congenital muscular dystrophy in a Sudanese family. A case report |
title_sort | rare variant in lama2 gene causing congenital muscular dystrophy in a sudanese family. a case report |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598405/ https://www.ncbi.nlm.nih.gov/pubmed/31309178 |
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