Literature DB >> 30426380

Comparison of clinical parameters with whole exome sequencing analysis results of autosomal recessive patients; a center experience.

M Elmas1, H Yıldız2, M Erdoğan3, B Gogus2, K Avcı2, M Solak2.   

Abstract

Whole-exome sequencing (WES) is an ideal method for the diagnosis of autosomal recessive diseases. The aim of this study was to evaluate the diagnostic power of WES in patients with autosomal recessive inheritance and to determine the relationship between genotype and phenotype. Retrospective screenings of 24 patients analysed with WES were performed and clinical and genetic data were evaluated. Any pathogenic mutation that could explain the suspected disease in 4 patients was not identified. A homozygous pathogenic mutation was detected in 18 patients. 2 patients had heterozygous mutations. According to this study results, WES is a successful technique to be used at the stage of diagnosis in patients who are accompanied by various degrees of intellectual disability matching the inheritance of the autosomal recessive.

Entities:  

Keywords:  Consanguinity; Intellectual disability; Microcephaly; Whole exome sequencing

Mesh:

Year:  2018        PMID: 30426380     DOI: 10.1007/s11033-018-4470-7

Source DB:  PubMed          Journal:  Mol Biol Rep        ISSN: 0301-4851            Impact factor:   2.316


  2 in total

1.  Novel Mutations in the GTPBP3 Gene for Mitochondrial Disease and Characteristics of Related Phenotypic Spectrum: The First Three Cases From China.

Authors:  Hui-Ming Yan; Zhi-Mei Liu; Bei Cao; Victor Wei Zhang; Yi-Duo He; Zheng-Jun Jia; Hui Xi; Jing Liu; Fang Fang; Hua Wang
Journal:  Front Genet       Date:  2021-07-01       Impact factor: 4.599

2.  Causative variant profile of collagen VI-related dystrophy in Japan.

Authors:  Michio Inoue; Yoshihiko Saito; Takahiro Yonekawa; Megumu Ogawa; Aritoshi Iida; Ichizo Nishino; Satoru Noguchi
Journal:  Orphanet J Rare Dis       Date:  2021-06-24       Impact factor: 4.123

  2 in total

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