Literature DB >> 35151641

A novel JAG1 frameshift variant causing Alagille syndrome with incomplete penetrance.

Yang Yang1, Hao Wang2.   

Abstract

OBJECTIVE: We report a novel frameshift variant of JAG1 in the prenatal setting, associated with Alagille syndrome1 with incomplete penetrance in a Chinese family. CASE REPORT: A pregnant woman was referred to our center due to fetal cardiovascular abnormality. Whole exome sequencing was employed for this family. A novel heterozygous maternal-origin frameshift variant c.1794_1797del in JAG1 was identified in the fetus. The mother and the fetus had the identical variant, but they demonstrated different clinical manifestations. The fetus was diagnosed with Alagille syndrome 1.
CONCLUSION: A novel frameshift variant in JAG1 was detected in a fetus diagnosed with Alagille syndrome 1, and it showed incomplete penetrance in the family.
Copyright © 2022 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Alagille syndrome; JAG1; Prenatal diagnosis; Whole exome sequencing

Mesh:

Substances:

Year:  2022        PMID: 35151641     DOI: 10.1016/j.clinbiochem.2022.02.004

Source DB:  PubMed          Journal:  Clin Biochem        ISSN: 0009-9120            Impact factor:   3.281


  1 in total

Review 1.  Pediatric acute viral hepatitis with atypical variants: Clinical dilemmas and natural history.

Authors:  Moinak Sen Sarma; Aathira Ravindranath
Journal:  World J Hepatol       Date:  2022-05-27
  1 in total

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