Case Report

Volume: 5 | Issue: 2 | Published: Dec 03, 2022 | Pages: 043 - 047 | DOI: 10.24911/JBCGenetics/183-1656681352

A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing


Authors: Gulhan Gurel orcid logo , Muhsin Elmas orcid logo , Basak Gogus orcid logo


Article Info

Authors

Gulhan Gurel

Department of Dermatology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar, Turkey

orcid logo ORCID

Muhsin Elmas

Department of Medical Genetics, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar, Turkey

orcid logo ORCID

Basak Gogus

Department of Medical Genetics, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar, Turkey.

orcid logo ORCID

Publication History

Received: July 01, 2022

Revised: November 10, 2022

Accepted: November 15, 2022

Published: December 03, 2022


Abstract


Background: The "autosomal recessive congenital ichthyosis (ARCI)" refers to a group of rare, heterogeneous, and non-syndromic disorders of keratinization, represented as abnormal scales over the entire body and attributable to defective epidermal keratinocyte differentiation and lipid metabolism. ARCI is caused by mutations in a wide variety of genes, including ABCA12, ALOX12B, ALOXE3, CYP4F22, NIPAL4, TGM1, CERS3, PNPLA1, CASP14, SDR9C7, and SULT2B1. The most common cause of ARCI is a TGM1 gene mutation, which is strongly associated with a collodion membrane at birth. Case presentation: A 15-year-old male patient presented with extensive scaling over the entire body since birth. His history revealed that he was born ash-colored in a membrane, kept in an incubator for one month, and clinically diagnosed with ichthyosis at birth. The patient, who had undergone no previous genetic testing, was subjected to whole exome sequencing with the preliminary diagnosis of autosomal recessive/X-linked recessive congenital ichthyosis. The analysis identified a homozygous c.1020delG change in the TGM1 gene in the form of a frameshift mutation that is classified as pathogenic according to the American College of Medical Genetics criteria. Conclusion: Next-generation sequencing technologies employing whole-exome sequencing enable the sequencing of all protein-coding DNA regions in a single run.

Keywords: Congenital, ichthyosis, TGM1, whole exome sequencing


Pubmed Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus. A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing. JBC Genetics. 2022; 03 (December 2022): 043-047. doi:10.24911/JBCGenetics/183-1656681352

Web Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus. A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing. https://www.jbcgenetics.com/index.php/articles/2107 [Access: April 27, 2025]. doi:10.24911/JBCGenetics/183-1656681352

AMA (American Medical Association) Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus. A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing. JBC Genetics. 2022; 03 (December 2022): 043-047. doi:10.24911/JBCGenetics/183-1656681352

Vancouver/ICMJE Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus. A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing. JBC Genetics. (2022), [cited April 27, 2025]; 03 (December 2022): 043-047. doi:10.24911/JBCGenetics/183-1656681352

Harvard Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus (2022) A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing. JBC Genetics, 03 (December 2022): 043-047. doi:10.24911/JBCGenetics/183-1656681352

Chicago Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus. "A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing." 03 (2022), 043-047. doi:10.24911/JBCGenetics/183-1656681352

MLA (The Modern Language Association) Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus. "A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing." 03.December 2022 (2022), 043-047. Print. doi:10.24911/JBCGenetics/183-1656681352

APA (American Psychological Association) Style

Gulhan Gurel, Muhsin Elmas, Basak Gogus (2022) A case of autosomal recessive congenital ichthyosis with a novel mutation identified in the TGM1 gene by whole exome sequencing. , 03 (December 2022), 043-047. doi:10.24911/JBCGenetics/183-1656681352