Case Report

Volume: 5 | Issue: 1 | Published: May 30, 2022 | Pages: 25 - 28 | DOI: 10.24911/JBCGenetics/183-1631602838

A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism


Authors: Khloud M. Al Rubaya orcid logo , Faten F. AlMijmaj orcid logo , Talal S. AlAnzi orcid logo , Abdullah A. AlJasser orcid logo


Article Info

Authors

Khloud M. Al Rubaya

Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi Arabia

orcid logo ORCID

Faten F. AlMijmaj

Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi Arabia

orcid logo ORCID

Talal S. AlAnzi

Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi Arabia

orcid logo ORCID

Abdullah A. AlJasser

Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

orcid logo ORCID

Publication History

Received: October 26, 2021

Revised: April 24, 2022

Accepted: May 09, 2022

Published: May 30, 2022


Abstract


Background: Isolated hypoparathyroidism comprises a set of heterogeneous inherited diseases associated with abnormal calcium metabolism exclusively due to parathyroid hormone (PTH) deficiency. Isolated hypoparathyroidism can be either sporadic or inherited. Genetic causes that impair the synthesis or secretion of PTH, such as calcium-sensing receptor and PTH defects, or defects in the development of the parathyroid gland [glial cell missing 2, (GCM2)], have been established as causes of familial isolated hypoparathyroidism. Transcription factor GCM2 is a crucial regulator of parathyroid gland homeostasis. Transmission of pathogenic variants encoding GCM2 occurs in an autosomal recessive or dominant manner. Case Presentation: Herein, we describe the case of a 12-year-old boy, born to consanguineous parents, who presented with abnormal movement during the first week of birth. Laboratory results revealed hypocalcemia, hyperphosphatemia, and low PTH levels. Genetic testing detected a novel homozygous variant in the GCM2 gene, c.391C>T (p.Arg131*). Although this variant has not been previously described, it is likely the pathogenic cause of this condition. Conclusion: To the best of authors' knowledge, this variant has not been listed in any database. Proper replacement therapy is likely to have good long-term outcomes for our patient.

Keywords: GCM2, GCMB, hypoparathyroidism, c.391C>T, CASR, case report


Pubmed Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser. A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism. JBC Genetics. 2022; 30 (May 2022): 25-28. doi:10.24911/JBCGenetics/183-1631602838

Web Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser. A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism. https://www.jbcgenetics.com/index.php/articles/2103 [Access: April 27, 2025]. doi:10.24911/JBCGenetics/183-1631602838

AMA (American Medical Association) Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser. A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism. JBC Genetics. 2022; 30 (May 2022): 25-28. doi:10.24911/JBCGenetics/183-1631602838

Vancouver/ICMJE Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser. A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism. JBC Genetics. (2022), [cited April 27, 2025]; 30 (May 2022): 25-28. doi:10.24911/JBCGenetics/183-1631602838

Harvard Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser (2022) A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism. JBC Genetics, 30 (May 2022): 25-28. doi:10.24911/JBCGenetics/183-1631602838

Chicago Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser. "A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism." 30 (2022), 25-28. doi:10.24911/JBCGenetics/183-1631602838

MLA (The Modern Language Association) Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser. "A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism." 30.May 2022 (2022), 25-28. Print. doi:10.24911/JBCGenetics/183-1631602838

APA (American Psychological Association) Style

Khloud M. Al Rubaya, Faten F. AlMijmaj, Talal S. AlAnzi, Abdullah A. AlJasser (2022) A novel GCM2 mutation identified in an infant with familial isolated hypoparathyroidism. , 30 (May 2022), 25-28. doi:10.24911/JBCGenetics/183-1631602838