Retrospective Study
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 16, 2023; 11(11): 2412-2422
Published online Apr 16, 2023. doi: 10.12998/wjcc.v11.i11.2412
Study of pathogenic genes in a pedigree with familial dilated cardiomyopathy
Xin-Ru Zhang, Hang Ren, Fang Yao, Yang Liu, Chun-Li Song
Xin-Ru Zhang, Department of Pharmacy, The Second Hospital of Jilin University, Changchun 130000, Jilin Province, China
Hang Ren, Fang Yao, Yang Liu, Chun-Li Song, Department of Cardiovascular Medicine, The Second Hospital of Jilin University, Changchun 130000, Jilin Province, China
Author contributions: Zhang XR contributed to the concept, definition of intellectual content, literature search, clinical studies, statistical analysis, manuscript preparation and review; Song CL contributed to the design and manuscript editing; Liu Y contributed to the experimental studies; Yao F contributed to the data acquisition; Ren H contributed to the data analysis.
Supported by the Jilin Provincial Healthcare Talent Special Program, No. 2019SCZT08.
Institutional review board statement: The study was reviewed and approved by the Ethics Committee of The Second Hospital of Jilin University.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Chun-Li Song, MD, Chief Physician, Professor, Department of Cardiovascular Medicine, The Second Hospital of Jilin University, Ziqiang Street, Nanguan District, Changchun 130000, Jilin Province, China. songchunl@jlu.edu.cn
Received: November 12, 2022
Peer-review started: November 12, 2022
First decision: February 14, 2023
Revised: February 22, 2023
Accepted: March 15, 2023
Article in press: March 15, 2023
Published online: April 16, 2023
Processing time: 145 Days and 3 Hours
ARTICLE HIGHLIGHTS
Research background

Dilated cardiomyopathy (DCM) is a type of genetically heterogeneous cardiomyopathy; it is the primary cause of heart transplantation and the third most common cause of heart failure, malignant arrhythmia, and sudden death. Although DCM has obvious genetic heterogeneity, with nearly 50% of DCM cases caused by genetic factors that are dominant in pathogenesis, research on the pathogenic genes associated with DCM is lacking.

Research motivation

With the development of next-generation sequencing technology, it has recently been found that DCM is related to variations in a number of genes encoding sarcomeric, cytoskeletal, nuclear membrane, and desmosomal proteins. However, only ~40% of familial DCM patients harbor known hereditary changes in pathogenic genes, while the etiology of the remaining 60% of familial DCM patients remains unclear. Therefore, the identification of pathogenic genes in DCM through pedigree analysis has become critical in the field of cardiovascular disease.

Research objectives

Our research team identified a typical DCM pedigree clinically. This study aimed to identify pathogenic genes in DCM through pedigree analysis.

Research methods

Our research team sequenced the whole exomes of seven samples in the pedigree using high-throughput sequencing technology, namely next-generation sequencing, and verified the potential candidate gene mutations in nineteen samples of the pedigree using Sanger sequencing. Then, we identified the sample sequence mutations using bioinformatics methods, and identified the family pathogenic gene mutations according to the family sample clinical information and gene variation annotation information.

Research results

A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered. The codon changes to c.T9199C and the amino acid changes to p.F3067L (phenylalanine mutates to leucine). It is a non-synonymous mutation (i.e., missense mutation).

Research conclusions

The mutation-ANK2p.F3067L was completely consistent with the clinical information for this DCM pedigree. It is considered a novel and potentially pathogenic gene mutation in DCM.

Research perspectives

Our study adds new members to DCM pathogenic genes, and makes a significant contribution to the literature. It would be useful for clinical implications of these results in medicine, especially general medicine.