Literature DB >> 32162566

A Novel Nonsense FMN2 Mutation in Nonsyndromic Autosomal Recessive Intellectual Disability Syndrome.

Orhan Gorukmez1, Ozlem Gorukmez1, Arzu Ekici2.   

Abstract

Introduction Genetic causes of the intellectual disability Nonsyndromic Autosomal-Recessive Intellectual Disability Syndrome (MRT47, MIM 616193) are mutations in the recently described FMN2 (formin 2 gene). Case report: A boy with intellectual disability had a novel homozygous nonsense mutation (c.2245C > T/p.Gln749*) leading to a premature stop codon in exon 6 of the FMN2 (NM_001305424) gene detected by Clinical Exome Sequencing (CES).
Conclusion: Clinical features of a patient with a novel nonsense FMN2 mutation is presented. We urge the change in the OMIM nomenclature from Mental Retardation, Autosomal Recessive 47 (MRT47, MIM 616193) to 'Nonsyndromic Autosomal-Recessive Intellectual Disability Syndrome'.

Entities:  

Keywords:  CES; FMN2; ID; novel mutation

Mesh:

Substances:

Year:  2020        PMID: 32162566     DOI: 10.1080/15513815.2020.1737991

Source DB:  PubMed          Journal:  Fetal Pediatr Pathol        ISSN: 1551-3815            Impact factor:   0.958


  3 in total

1.  The Formin Fmn2b Is Required for the Development of an Excitatory Interneuron Module in the Zebrafish Acoustic Startle Circuit.

Authors:  Dhriti Nagar; Tomin K James; Ratnakar Mishra; Shrobona Guha; Shawn M Burgess; Aurnab Ghose
Journal:  eNeuro       Date:  2021-07-09

Review 2.  Formins in Human Disease.

Authors:  Leticia Labat-de-Hoz; Miguel A Alonso
Journal:  Cells       Date:  2021-09-27       Impact factor: 6.600

3.  Heterozygous FMN2 missense variant found in a family case of premature ovarian insufficiency.

Authors:  Jie Li; Tianliu Peng; Le Wang; Panpan Long; Ruping Quan; Hangjing Tan; Minghua Zeng; Xue Wu; Junting Yang; Hongmei Xiao; Xiaobo Shi
Journal:  J Ovarian Res       Date:  2022-02-28       Impact factor: 4.234

  3 in total

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