Literature DB >> 33420945

Small mutations in Duchenne/Becker muscular dystrophy in 164 unrelated Polish patients.

Janusz G Zimowski1,2, Joanna Purzycka3, Magdalena Pawelec3, Katarzyna Ozdarska3, Jacek Zaremba3.   

Abstract

In the 164 patients with Duchenne/Becker muscular dystrophy, we found 142 different small mutations including 51 novel mutations not listed in the LOVD, the UMD-DMD, the ClinVar, and the HGMD databases. Among all mutations, nonsense mutations occurred in 45.7%, frameshift mutations in 32.9%, and splicing mutations in 19.5%. Small mutations were distributed throughout the whole dystrophin gene. Splicing mutations were twice more common in BMD patients than in DMD patients. Eighty-two percent of mothers of the males affected with DMD/BMD were found to be carriers of small mutations.

Entities:  

Keywords:  BMD; Carriers; DMD; Novel mutations; Point mutations; Small mutations

Year:  2021        PMID: 33420945     DOI: 10.1007/s13353-020-00605-0

Source DB:  PubMed          Journal:  J Appl Genet        ISSN: 1234-1983            Impact factor:   3.240


  20 in total

1.  DMD exon 1 truncating point mutations: amelioration of phenotype by alternative translation initiation in exon 6.

Authors:  Olga L Gurvich; Baijayanta Maiti; Robert B Weiss; Gaurav Aggarwal; Michael T Howard; Kevin M Flanigan
Journal:  Hum Mutat       Date:  2009-04       Impact factor: 4.878

2.  DMD mutation spectrum analysis in 613 Chinese patients with dystrophinopathy.

Authors:  Ruolan Guo; Guosheng Zhu; Huimin Zhu; Ruiyu Ma; Ying Peng; Desheng Liang; Lingqian Wu
Journal:  J Hum Genet       Date:  2015-05-14       Impact factor: 3.172

3.  Protein- and mRNA-based phenotype-genotype correlations in DMD/BMD with point mutations and molecular basis for BMD with nonsense and frameshift mutations in the DMD gene.

Authors:  Nathalie Deburgrave; Fatma Daoud; Stéphane Llense; Jean Claude Barbot; Dominique Récan; Cécile Peccate; Arthur H M Burghes; Christophe Béroud; Luis Garcia; Jean-Claude Kaplan; Jamel Chelly; France Leturcq
Journal:  Hum Mutat       Date:  2007-02       Impact factor: 4.878

4.  Consecutive analysis of mutation spectrum in the dystrophin gene of 507 Korean boys with Duchenne/Becker muscular dystrophy in a single center.

Authors:  Anna Cho; Moon-Woo Seong; Byung Chan Lim; Hwa Jeen Lee; Jung Hye Byeon; Seung Soo Kim; Soo Yeon Kim; Sun Ah Choi; Ai-Lynn Wong; Jeongho Lee; Jon Soo Kim; Hye Won Ryu; Jin Sook Lee; Hunmin Kim; Hee Hwang; Ji Eun Choi; Ki Joong Kim; Young Seung Hwang; Ki Ho Hong; Seungman Park; Sung Im Cho; Seung Jun Lee; Hyunwoong Park; Soo Hyun Seo; Sung Sup Park; Jong Hee Chae
Journal:  Muscle Nerve       Date:  2017-01-20       Impact factor: 3.217

5.  Nonsense mutation-associated Becker muscular dystrophy: interplay between exon definition and splicing regulatory elements within the DMD gene.

Authors:  Kevin M Flanigan; Diane M Dunn; Andrew von Niederhausern; Payam Soltanzadeh; Michael T Howard; Jacinda B Sampson; Kathryn J Swoboda; Mark B Bromberg; Jerry R Mendell; Laura E Taylor; Christine B Anderson; Alan Pestronk; Julaine M Florence; Anne M Connolly; Katherine D Mathews; Brenda Wong; Richard S Finkel; Carsten G Bonnemann; John W Day; Craig McDonald; Robert B Weiss
Journal:  Hum Mutat       Date:  2011-03       Impact factor: 4.878

6.  Integrated DNA, cDNA, and protein studies in Becker muscular dystrophy show high exception to the reading frame rule.

Authors:  Akanchha Kesari; Laura N Pirra; Lakshmi Bremadesam; Orinthal McIntyre; Erynn Gordon; Alberto L Dubrovsky; V Viswanathan; Eric P Hoffman
Journal:  Hum Mutat       Date:  2008-05       Impact factor: 4.878

7.  Complete cloning of the Duchenne muscular dystrophy (DMD) cDNA and preliminary genomic organization of the DMD gene in normal and affected individuals.

Authors:  M Koenig; E P Hoffman; C J Bertelson; A P Monaco; C Feener; L M Kunkel
Journal:  Cell       Date:  1987-07-31       Impact factor: 41.582

8.  Mutational spectrum of DMD mutations in dystrophinopathy patients: application of modern diagnostic techniques to a large cohort.

Authors:  Kevin M Flanigan; Diane M Dunn; Andrew von Niederhausern; Payam Soltanzadeh; Eduard Gappmaier; Michael T Howard; Jacinda B Sampson; Jerry R Mendell; Cheryl Wall; Wendy M King; Alan Pestronk; Julaine M Florence; Anne M Connolly; Katherine D Mathews; Carrie M Stephan; Karla S Laubenthal; Brenda L Wong; Paula J Morehart; Amy Meyer; Richard S Finkel; Carsten G Bonnemann; Livija Medne; John W Day; Joline C Dalton; Marcia K Margolis; Veronica J Hinton; Robert B Weiss
Journal:  Hum Mutat       Date:  2009-12       Impact factor: 4.878

9.  On the origin of deletions and point mutations in Duchenne muscular dystrophy: most deletions arise in oogenesis and most point mutations result from events in spermatogenesis.

Authors:  T Grimm; G Meng; S Liechti-Gallati; T Bettecken; C R Müller; B Müller
Journal:  J Med Genet       Date:  1994-03       Impact factor: 6.318

Review 10.  Exon-skipping advances for Duchenne muscular dystrophy.

Authors:  Lucía Echevarría; Philippine Aupy; Aurélie Goyenvalle
Journal:  Hum Mol Genet       Date:  2018-08-01       Impact factor: 6.150

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