Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: AMIN, MUTAZ, BAKHIT, YOUSUF, KOKO, MAHMOUD, IBRAHIM, MOHAMED OSAMA MIRGAHNI, SALIH, MA, IBRAHIM, MUNTASER, SEIDI, OSHEIK A
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Pacini Editore srl 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598405/
https://www.ncbi.nlm.nih.gov/pubmed/31309178
_version_ 1783430766868299776
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
work_keys_str_mv AT aminmutaz rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport
AT bakhityousuf rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport
AT kokomahmoud rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport
AT ibrahimmohamedosamamirgahni rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport
AT salihma rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport
AT ibrahimmuntaser rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport
AT seidiosheika rarevariantinlama2genecausingcongenitalmusculardystrophyinasudanesefamilyacasereport