Novel insight into FCSK‐congenital disorder of glycosylation through a CRISPR‐generated cell model

Background FCSK‐congenital disorder of glycosylation (FCSK‐CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe mul...

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Vydané v:Molecular genetics & genomic medicine Ročník 12; číslo 5; s. e2445 - n/a
Hlavní autori: Fazelzadeh Haghighi, Maryam, Jafari Khamirani, Hossein, Fallahi, Jafar, Monfared, Ali Arabi, Ashrafi Dehkordi, Korosh, Tabei, Seyed Mohammad Bagher
Médium: Journal Article
Jazyk:English
Vydavateľské údaje: United States John Wiley & Sons, Inc 01.05.2024
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Abstract Background FCSK‐congenital disorder of glycosylation (FCSK‐CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK‐CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency. Methods In this study, CRISPR/Cas9 was employed to construct an FCSK‐CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real‐time PCR analyses. Results Comparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O‐fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF‐like repeats O‐fucosylation. Conclusion This study expands insight into the FCSK‐CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease. The current study exploring FCSK‐CDG through a CRISPR‐generated cell model demonstrated the effect of this disease on EGF‐like repeats O‐fucosylation by revealing NOTCH3 as the first identified gene whose expression level alters due to FCSK‐CDG. In addition, it suggested a probable effect of this disease on secretory glycoproteins.
AbstractList The current study exploring FCSK‐CDG through a CRISPR‐generated cell model demonstrated the effect of this disease on EGF‐like repeats O‐fucosylation by revealing NOTCH3 as the first identified gene whose expression level alters due to FCSK‐CDG. In addition, it suggested a probable effect of this disease on secretory glycoproteins.
BackgroundFCSK-congenital disorder of glycosylation (FCSK-CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK-CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency.MethodsIn this study, CRISPR/Cas9 was employed to construct an FCSK-CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real-time PCR analyses.ResultsComparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O-fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF-like repeats O-fucosylation.ConclusionThis study expands insight into the FCSK-CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease.
FCSK-congenital disorder of glycosylation (FCSK-CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK-CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency.BACKGROUNDFCSK-congenital disorder of glycosylation (FCSK-CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK-CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency.In this study, CRISPR/Cas9 was employed to construct an FCSK-CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real-time PCR analyses.METHODSIn this study, CRISPR/Cas9 was employed to construct an FCSK-CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real-time PCR analyses.Comparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O-fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF-like repeats O-fucosylation.RESULTSComparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O-fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF-like repeats O-fucosylation.This study expands insight into the FCSK-CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease.CONCLUSIONThis study expands insight into the FCSK-CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease.
Background FCSK‐congenital disorder of glycosylation (FCSK‐CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK‐CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency. Methods In this study, CRISPR/Cas9 was employed to construct an FCSK‐CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real‐time PCR analyses. Results Comparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O‐fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF‐like repeats O‐fucosylation. Conclusion This study expands insight into the FCSK‐CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease. The current study exploring FCSK‐CDG through a CRISPR‐generated cell model demonstrated the effect of this disease on EGF‐like repeats O‐fucosylation by revealing NOTCH3 as the first identified gene whose expression level alters due to FCSK‐CDG. In addition, it suggested a probable effect of this disease on secretory glycoproteins.
Abstract Background FCSK‐congenital disorder of glycosylation (FCSK‐CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK‐CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency. Methods In this study, CRISPR/Cas9 was employed to construct an FCSK‐CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real‐time PCR analyses. Results Comparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O‐fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF‐like repeats O‐fucosylation. Conclusion This study expands insight into the FCSK‐CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease.
FCSK-congenital disorder of glycosylation (FCSK-CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to mutations in the fucokinase encoding gene, FCSK. Despite the essential role of fucokinase in the fucose salvage pathway and severe multisystem manifestations of FCSK-CDG patients, it is not elucidated which cells or which types of fucosylation are affected by its deficiency. In this study, CRISPR/Cas9 was employed to construct an FCSK-CDG cell model and explore the molecular mechanisms of the disease by lectin flow cytometry and real-time PCR analyses. Comparison of cellular fucosylation by lectin flow cytometry in the created CRISPR/Cas9 FCSK knockout and the same unedited cell lines showed no significant change in the amount of cell surface fucosylated glycans, which is consistent with the only documented previous study on different cell types. It suggests a probable effect of this disease on secretory glycoproteins. Investigating O-fucosylation by analysis of the NOTCH3 gene expression as a potential target revealed a significant decrease in the FCSK knockout cells compared with the same unedited ones, proving the effect of fucokinase deficiency on EGF-like repeats O-fucosylation. This study expands insight into the FCSK-CDG molecular mechanism; to the best of our knowledge, it is the first research conducted to reveal a gene whose expression level alters due to this disease.
Author Tabei, Seyed Mohammad Bagher
Fallahi, Jafar
Fazelzadeh Haghighi, Maryam
Jafari Khamirani, Hossein
Ashrafi Dehkordi, Korosh
Monfared, Ali Arabi
AuthorAffiliation 1 Department of Molecular Medicine, School of Advanced Technologies Shahrekord University of Medical Sciences Shahrekord Iran
5 Maternal‐Fetal Medicine Research Center Shiraz University of Medical Sciences Shiraz Iran
2 Department of Medical Genetics Shiraz University of Medical Sciences Shiraz Iran
3 Molecular Medicine Department, School of Advanced Medical Sciences and Technologies Shiraz University of Medical Sciences Shiraz Iran
4 Central Research Laboratory Shiraz University of Medical Sciences Shiraz Iran
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Keywords congenital disorder of glycosylation
CRISPR/Cas9
FCSK
fucokinase
Language English
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Notes Seyed Mohammad Bagher Tabei and Korosh Ashrafi Dehkordi should be considered Joint senior author.
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SSID ssj0000884167
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Snippet Background FCSK‐congenital disorder of glycosylation (FCSK‐CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation...
FCSK-congenital disorder of glycosylation (FCSK-CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation due to...
BackgroundFCSK-congenital disorder of glycosylation (FCSK-CDG) is a recently discovered rare autosomal recessive genetic disorder with defective fucosylation...
The current study exploring FCSK‐CDG through a CRISPR‐generated cell model demonstrated the effect of this disease on EGF‐like repeats O‐fucosylation by...
Abstract Background FCSK‐congenital disorder of glycosylation (FCSK‐CDG) is a recently discovered rare autosomal recessive genetic disorder with defective...
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pubmedcentral
proquest
pubmed
crossref
wiley
SourceType Open Website
Open Access Repository
Aggregation Database
Index Database
Publisher
StartPage e2445
SubjectTerms Age
Cell culture
Cell lines
Cell surface
Congenital anomalies
Congenital diseases
congenital disorder of glycosylation
Congenital Disorders of Glycosylation - genetics
Congenital Disorders of Glycosylation - metabolism
Congenital Disorders of Glycosylation - pathology
Convulsions & seizures
CRISPR
CRISPR-Cas Systems
CRISPR/Cas9
Design
Disease
Enzymes
Epilepsy
FCSK
Flow cytometry
Fucokinase
Fucose - metabolism
Gene expression
Genetic disorders
Glycoproteins
Glycosylation
Hereditary diseases
Humans
Intellectual disabilities
Molecular modelling
Notch3 protein
Original
Patients
Phosphotransferases (Alcohol Group Acceptor)
Polysaccharides
Receptors, Notch - genetics
Receptors, Notch - metabolism
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  priority: 102
  providerName: Wiley-Blackwell
Title Novel insight into FCSK‐congenital disorder of glycosylation through a CRISPR‐generated cell model
URI https://onlinelibrary.wiley.com/doi/abs/10.1002%2Fmgg3.2445
https://www.ncbi.nlm.nih.gov/pubmed/38722107
https://www.proquest.com/docview/3060735819
https://www.proquest.com/docview/3053972739
https://pubmed.ncbi.nlm.nih.gov/PMC11080630
https://doaj.org/article/7ccb8cb61b94404a93e9180efbf1e4a7
Volume 12
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