Literature DB >> 35037378

Werner syndrome in a Lebanese family.

Batoul Jaafar1, Mona Nasrallah1, Bianca Sievers2, Junko Oshima3, Davor Lessel2.   

Abstract

Werner syndrome (WS) is an extremely rare, autosomal recessive segmental progeroid disorder caused by biallelic pathogenic variants in the WRN, which encodes a multifunctional nuclear protein that belongs to the RecQ family of DNA helicases. Despite extensive research on WS in the last years, the population-specific mutational spectrum still needs to be elucidated. Moreover, there is an evident lack of detailed clinical descriptions accompanied with photographs of affected individuals. Here, we report a consanguineous Lebanese family in whom we identified a pathogenic homozygous nonsense variant c.1111G>T, p.Glu371* in the WRN. The index individual, at the age of 54 years, was suspected to have WS due to a history of early-onset cataracts, premature hair loss and graying, chronic nonhealing leg ulcers, Achilles' tendon calcifications, type 2 diabetes mellitus, dyslipidemia, hypothyroidism, and premature coronary artery disease. His four sisters, three of which deceased in the fifth decade, had clinical signs suggestive of WS. Moreover, his daughter, aged 23 years, had short stature, hair loss and flat feet. Taken together, we report a detailed clinical course of disease in several affected members of a consanguineous family, which is additionally documented by photographs.
© 2022 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.

Entities:  

Keywords:  Werner helicase; Werner syndrome; premature aging syndrome; segmental progeroid syndrome

Mesh:

Substances:

Year:  2022        PMID: 35037378      PMCID: PMC8995352          DOI: 10.1002/ajmg.a.62654

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  11 in total

1.  Spectral karyotyping of Werner syndrome fibroblast cultures.

Authors:  R Melcher; R von Golitschek; C Steinlein; D Schindler; H Neitzel; K Kainer; M Schmid; H Hoehn
Journal:  Cytogenet Cell Genet       Date:  2000

2.  WRN mutations in Werner syndrome patients: genomic rearrangements, unusual intronic mutations and ethnic-specific alterations.

Authors:  Katrin Friedrich; Lin Lee; Dru F Leistritz; Gudrun Nürnberg; Bidisha Saha; Fuki M Hisama; Daniel K Eyman; Davor Lessel; Peter Nürnberg; Chumei Li; María J Garcia-F-Villalta; Carolien M Kets; Joerg Schmidtke; Vítor Tedim Cruz; Peter C Van den Akker; Joseph Boak; Dincy Peter; Goli Compoginis; Kivanc Cefle; Sukru Ozturk; Norberto López; Theda Wessel; Martin Poot; P F Ippel; Birgit Groff-Kellermann; Holger Hoehn; George M Martin; Christian Kubisch; Junko Oshima
Journal:  Hum Genet       Date:  2010-05-05       Impact factor: 4.132

3.  Werner syndrome and mutations of the WRN and LMNA genes in France.

Authors:  Nancy A Uhrhammer; Laurence Lafarge; Laetitia Dos Santos; Anna Domaszewska; Magdalena Lange; Yong Yang; Selim Aractingi; Didier Bessis; Yves-Jean Bignon
Journal:  Hum Mutat       Date:  2006-07       Impact factor: 4.878

4.  Homozygous and compound heterozygous mutations at the Werner syndrome locus.

Authors:  J Oshima; C E Yu; C Piussan; G Klein; J Jabkowski; S Balci; T Miki; J Nakura; T Ogihara; J Ells; M Smith; M I Melaragno; M Fraccaro; S Scappaticci; J Matthews; S Ouais; A Jarzebowicz; G D Schellenberg; G M Martin
Journal:  Hum Mol Genet       Date:  1996-12       Impact factor: 6.150

5.  Excess of rare cancers in Werner syndrome (adult progeria).

Authors:  M Goto; R W Miller; Y Ishikawa; H Sugano
Journal:  Cancer Epidemiol Biomarkers Prev       Date:  1996-04       Impact factor: 4.254

6.  Unusual features of thyroid carcinomas in Japanese patients with Werner syndrome and possible genotype-phenotype relations to cell type and race.

Authors:  Y Ishikawa; H Sugano; T Matsumoto; Y Furuichi; R W Miller; M Goto
Journal:  Cancer       Date:  1999-03-15       Impact factor: 6.860

7.  Diverged nuclear localization of Werner helicase in human and mouse cells.

Authors:  T Suzuki; M Shiratori; Y Furuichi; T Matsumoto
Journal:  Oncogene       Date:  2001-05-03       Impact factor: 9.867

8.  Hierarchical deterioration of body systems in Werner's syndrome: implications for normal ageing.

Authors:  M Goto
Journal:  Mech Ageing Dev       Date:  1997-12       Impact factor: 5.432

Review 9.  WRN Mutation Update: Mutation Spectrum, Patient Registries, and Translational Prospects.

Authors:  Koutaro Yokote; Sirisak Chanprasert; Lin Lee; Katharina Eirich; Minoru Takemoto; Aki Watanabe; Naoko Koizumi; Davor Lessel; Takayasu Mori; Fuki M Hisama; Paula D Ladd; Brad Angle; Hagit Baris; Kivanc Cefle; Sukru Palanduz; Sukru Ozturk; Antoinette Chateau; Kentaro Deguchi; T K M Easwar; Antonio Federico; Amy Fox; Theresa A Grebe; Beverly Hay; Sheela Nampoothiri; Karen Seiter; Elizabeth Streeten; Raul E Piña-Aguilar; Gemma Poke; Martin Poot; Renata Posmyk; George M Martin; Christian Kubisch; Detlev Schindler; Junko Oshima
Journal:  Hum Mutat       Date:  2016-10-07       Impact factor: 4.878

10.  Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.

Authors:  Sue Richards; Nazneen Aziz; Sherri Bale; David Bick; Soma Das; Julie Gastier-Foster; Wayne W Grody; Madhuri Hegde; Elaine Lyon; Elaine Spector; Karl Voelkerding; Heidi L Rehm
Journal:  Genet Med       Date:  2015-03-05       Impact factor: 8.822

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