Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type

Background: We aimed to analyze mutations of the pathogenic gene in dyssegmental dysplasia Silverman-Handmaker (DDSH) type associated with the Heparin sulfate proteoglycan 2 (HSPG2) gene. Case: Prenatal testing for genetic mutations associated with fetal DDSH were performed on a pregnant woman with...

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Main Authors: Yunxia Wang, Hui Wang
Format: Article
Language:English
Published: IMR Press 2022-02-01
Series:Clinical and Experimental Obstetrics & Gynecology
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Online Access:https://www.imrpress.com/journal/CEOG/49/2/10.31083/j.ceog4902037
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author Yunxia Wang
Hui Wang
author_facet Yunxia Wang
Hui Wang
author_sort Yunxia Wang
collection DOAJ
description Background: We aimed to analyze mutations of the pathogenic gene in dyssegmental dysplasia Silverman-Handmaker (DDSH) type associated with the Heparin sulfate proteoglycan 2 (HSPG2) gene. Case: Prenatal testing for genetic mutations associated with fetal DDSH were performed on a pregnant woman with previous history of carrying a fetus with short limb malformation at the 17th week of gestation. DNA was extracted from amniotic fluid and next-generation sequencing-based deep panel sequencing was performed on the Illumina NextSeq platform to identify possible causative mutations of DDSH. Results: Two novel heterozygous mutations in HSPG2 gene, c.6001dupC (p. R2001pfs*19) and c.11207G>A (p. R373Q), were identified and associated with the DDSH diagnosis. Conclusion: This is the first report to prenatally identify novel mutations in HSPG2 that confirms a DDSH diagnosis.
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series Clinical and Experimental Obstetrics & Gynecology
spelling doaj-art-2f9bf69e7856464aa944f9da9c1ff4b02025-08-20T02:08:09ZengIMR PressClinical and Experimental Obstetrics & Gynecology0390-66632022-02-014923710.31083/j.ceog4902037S0390-6663(22)01686-4Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker typeYunxia Wang0Hui Wang1Department of Obstetrics, Shenzhen Futian Maternal and Child Health Hospital, 518045 Shenzhen, Guangdong, ChinaDepartment of Obstetrics, Shenzhen Maternal and Child Health Hospital, 518000 Shenzhen, Guangdong, ChinaBackground: We aimed to analyze mutations of the pathogenic gene in dyssegmental dysplasia Silverman-Handmaker (DDSH) type associated with the Heparin sulfate proteoglycan 2 (HSPG2) gene. Case: Prenatal testing for genetic mutations associated with fetal DDSH were performed on a pregnant woman with previous history of carrying a fetus with short limb malformation at the 17th week of gestation. DNA was extracted from amniotic fluid and next-generation sequencing-based deep panel sequencing was performed on the Illumina NextSeq platform to identify possible causative mutations of DDSH. Results: Two novel heterozygous mutations in HSPG2 gene, c.6001dupC (p. R2001pfs*19) and c.11207G>A (p. R373Q), were identified and associated with the DDSH diagnosis. Conclusion: This is the first report to prenatally identify novel mutations in HSPG2 that confirms a DDSH diagnosis.https://www.imrpress.com/journal/CEOG/49/2/10.31083/j.ceog4902037heparin sulfate proteoglycan 2dyssegmental dysplasia silverman-handmakergene mutationprenatal diagnosis
spellingShingle Yunxia Wang
Hui Wang
Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type
Clinical and Experimental Obstetrics & Gynecology
heparin sulfate proteoglycan 2
dyssegmental dysplasia silverman-handmaker
gene mutation
prenatal diagnosis
title Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type
title_full Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type
title_fullStr Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type
title_full_unstemmed Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type
title_short Prenatal identification of novel HSPG2 variants associated with dyssegmental dysplasia Silverman-Handmaker type
title_sort prenatal identification of novel hspg2 variants associated with dyssegmental dysplasia silverman handmaker type
topic heparin sulfate proteoglycan 2
dyssegmental dysplasia silverman-handmaker
gene mutation
prenatal diagnosis
url https://www.imrpress.com/journal/CEOG/49/2/10.31083/j.ceog4902037
work_keys_str_mv AT yunxiawang prenatalidentificationofnovelhspg2variantsassociatedwithdyssegmentaldysplasiasilvermanhandmakertype
AT huiwang prenatalidentificationofnovelhspg2variantsassociatedwithdyssegmentaldysplasiasilvermanhandmakertype