Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.

Chondroitin/dermatan sulfate (CS/DS) proteoglycans are indispensable for animal development and homeostasis but the large number of enzymes involved in their biosynthesis have made CS/DS function a challenging problem to study genetically. In our study, we generated loss-of-function alleles in zebra...

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Main Authors: Judith Habicher, Gaurav K Varshney, Laura Waldmann, Daniel Snitting, Amin Allalou, Hanqing Zhang, Abdurrahman Ghanem, Caroline Öhman Mägi, Tabea Dierker, Lena Kjellén, Shawn M Burgess, Johan Ledin
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2022-02-01
Series:PLoS Genetics
Online Access:https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1010067&type=printable
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author Judith Habicher
Gaurav K Varshney
Laura Waldmann
Daniel Snitting
Amin Allalou
Hanqing Zhang
Abdurrahman Ghanem
Caroline Öhman Mägi
Tabea Dierker
Lena Kjellén
Shawn M Burgess
Johan Ledin
author_facet Judith Habicher
Gaurav K Varshney
Laura Waldmann
Daniel Snitting
Amin Allalou
Hanqing Zhang
Abdurrahman Ghanem
Caroline Öhman Mägi
Tabea Dierker
Lena Kjellén
Shawn M Burgess
Johan Ledin
author_sort Judith Habicher
collection DOAJ
description Chondroitin/dermatan sulfate (CS/DS) proteoglycans are indispensable for animal development and homeostasis but the large number of enzymes involved in their biosynthesis have made CS/DS function a challenging problem to study genetically. In our study, we generated loss-of-function alleles in zebrafish genes encoding CS/DS biosynthetic enzymes and characterized the effect on development in single and double mutants. Homozygous mutants in chsy1, csgalnact1a, csgalnat2, chpfa, ust and chst7, respectively, develop to adults. However, csgalnact1a-/- fish develop distinct craniofacial defects while the chsy1-/- skeletal phenotype is milder and the remaining mutants display no gross morphological abnormalities. These results suggest a high redundancy for the CS/DS biosynthetic enzymes and to further reduce CS/DS biosynthesis we combined mutant alleles. The craniofacial phenotype is further enhanced in csgalnact1a-/-;chsy1-/- adults and csgalnact1a-/-;csgalnact2-/- larvae. While csgalnact1a-/-;csgalnact2-/- was the most affected allele combination in our study, CS/DS is still not completely abolished. Transcriptome analysis of chsy1-/-, csgalnact1a-/- and csgalnact1a-/-;csgalnact2-/- larvae revealed that the expression had changed in a similar way in the three mutant lines but no differential expression was found in any of fifty GAG biosynthesis enzymes identified. Thus, zebrafish larvae do not increase transcription of GAG biosynthesis genes as a consequence of decreased CS/DS biosynthesis. The new zebrafish lines develop phenotypes similar to clinical characteristics of several human congenital disorders making the mutants potentially useful to study disease mechanisms and treatment.
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spelling doaj-art-14829a228b8e40ef9f46f01aa8efbb7b2025-08-20T03:44:46ZengPublic Library of Science (PLoS)PLoS Genetics1553-73901553-74042022-02-01182e101006710.1371/journal.pgen.1010067Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.Judith HabicherGaurav K VarshneyLaura WaldmannDaniel SnittingAmin AllalouHanqing ZhangAbdurrahman GhanemCaroline Öhman MägiTabea DierkerLena KjellénShawn M BurgessJohan LedinChondroitin/dermatan sulfate (CS/DS) proteoglycans are indispensable for animal development and homeostasis but the large number of enzymes involved in their biosynthesis have made CS/DS function a challenging problem to study genetically. In our study, we generated loss-of-function alleles in zebrafish genes encoding CS/DS biosynthetic enzymes and characterized the effect on development in single and double mutants. Homozygous mutants in chsy1, csgalnact1a, csgalnat2, chpfa, ust and chst7, respectively, develop to adults. However, csgalnact1a-/- fish develop distinct craniofacial defects while the chsy1-/- skeletal phenotype is milder and the remaining mutants display no gross morphological abnormalities. These results suggest a high redundancy for the CS/DS biosynthetic enzymes and to further reduce CS/DS biosynthesis we combined mutant alleles. The craniofacial phenotype is further enhanced in csgalnact1a-/-;chsy1-/- adults and csgalnact1a-/-;csgalnact2-/- larvae. While csgalnact1a-/-;csgalnact2-/- was the most affected allele combination in our study, CS/DS is still not completely abolished. Transcriptome analysis of chsy1-/-, csgalnact1a-/- and csgalnact1a-/-;csgalnact2-/- larvae revealed that the expression had changed in a similar way in the three mutant lines but no differential expression was found in any of fifty GAG biosynthesis enzymes identified. Thus, zebrafish larvae do not increase transcription of GAG biosynthesis genes as a consequence of decreased CS/DS biosynthesis. The new zebrafish lines develop phenotypes similar to clinical characteristics of several human congenital disorders making the mutants potentially useful to study disease mechanisms and treatment.https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1010067&type=printable
spellingShingle Judith Habicher
Gaurav K Varshney
Laura Waldmann
Daniel Snitting
Amin Allalou
Hanqing Zhang
Abdurrahman Ghanem
Caroline Öhman Mägi
Tabea Dierker
Lena Kjellén
Shawn M Burgess
Johan Ledin
Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.
PLoS Genetics
title Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.
title_full Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.
title_fullStr Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.
title_full_unstemmed Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.
title_short Chondroitin/dermatan sulfate glycosyltransferase genes are essential for craniofacial development.
title_sort chondroitin dermatan sulfate glycosyltransferase genes are essential for craniofacial development
url https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1010067&type=printable
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