Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder

Gaucher disease (GD) is a metabolic disorder caused by mutations in the GBA1, located on 1q22. This gene encodes glucocerebrosidase (glucosylceramidase) enzyme. GD has a wide range of clinical manifestations from a perinatally lethal type to an asymptomatic form. While different types of targeted th...

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Main Authors: Mehran Reyhani-Ardabili, Mohadeseh Fathi, Soudeh Ghafouri-Fard
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Biochemistry and Biophysics Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S240558082400236X
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author Mehran Reyhani-Ardabili
Mohadeseh Fathi
Soudeh Ghafouri-Fard
author_facet Mehran Reyhani-Ardabili
Mohadeseh Fathi
Soudeh Ghafouri-Fard
author_sort Mehran Reyhani-Ardabili
collection DOAJ
description Gaucher disease (GD) is a metabolic disorder caused by mutations in the GBA1, located on 1q22. This gene encodes glucocerebrosidase (glucosylceramidase) enzyme. GD has a wide range of clinical manifestations from a perinatally lethal type to an asymptomatic form. While different types of targeted therapies and hematopoietic stem cell transplantation have been suggested as therapeutic options for patients with GD, success rates were not optimal. Recent advance in the CRISPR technology has raised the hope for treatment of metabolic disorders such as GD. This technology has also facilitated identification of the molecular mechanisms underlying pathologic events in this disorder. The current review addresses both mentioned aspects of application of CRISPR technology in the field of GD.
format Article
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publisher Elsevier
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series Biochemistry and Biophysics Reports
spelling doaj-art-b92f11cb75084d8e833919b789fbbc922025-08-20T02:19:51ZengElsevierBiochemistry and Biophysics Reports2405-58082024-12-014010187210.1016/j.bbrep.2024.101872Application of CRISPR/Cas9 technology in the modeling of Gaucher disorderMehran Reyhani-Ardabili0Mohadeseh Fathi1Soudeh Ghafouri-Fard2Department of Medical Genetics, Shahid Beheshti University of Medical Sciences, Tehran, IranDepartment of Medical Genetics, Shahid Beheshti University of Medical Sciences, Tehran, IranCorresponding author.; Department of Medical Genetics, Shahid Beheshti University of Medical Sciences, Tehran, IranGaucher disease (GD) is a metabolic disorder caused by mutations in the GBA1, located on 1q22. This gene encodes glucocerebrosidase (glucosylceramidase) enzyme. GD has a wide range of clinical manifestations from a perinatally lethal type to an asymptomatic form. While different types of targeted therapies and hematopoietic stem cell transplantation have been suggested as therapeutic options for patients with GD, success rates were not optimal. Recent advance in the CRISPR technology has raised the hope for treatment of metabolic disorders such as GD. This technology has also facilitated identification of the molecular mechanisms underlying pathologic events in this disorder. The current review addresses both mentioned aspects of application of CRISPR technology in the field of GD.http://www.sciencedirect.com/science/article/pii/S240558082400236XGaucher disease (GD)GBA1CRISPR
spellingShingle Mehran Reyhani-Ardabili
Mohadeseh Fathi
Soudeh Ghafouri-Fard
Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder
Biochemistry and Biophysics Reports
Gaucher disease (GD)
GBA1
CRISPR
title Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder
title_full Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder
title_fullStr Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder
title_full_unstemmed Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder
title_short Application of CRISPR/Cas9 technology in the modeling of Gaucher disorder
title_sort application of crispr cas9 technology in the modeling of gaucher disorder
topic Gaucher disease (GD)
GBA1
CRISPR
url http://www.sciencedirect.com/science/article/pii/S240558082400236X
work_keys_str_mv AT mehranreyhaniardabili applicationofcrisprcas9technologyinthemodelingofgaucherdisorder
AT mohadesehfathi applicationofcrisprcas9technologyinthemodelingofgaucherdisorder
AT soudehghafourifard applicationofcrisprcas9technologyinthemodelingofgaucherdisorder