Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype

Wolfram syndrome (WS), also known as a DIDMOAD (diabetes insipidus, early-onset diabetes mellitus, optic nerve atrophy and deafness) is a rare autosomal disorder caused by mutations in the Wolframin1 (<i>WFS1</i>) gene. Previous studies have revealed that glucagon-like peptide-1 receptor...

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Autores principales:	Toomas Jagomäe, Kadri Seppa, Riin Reimets, Marko Pastak, Mihkel Plaas, Miriam A. Hickey, Kaia Grete Kukker, Lieve Moons, Lies De Groef, Eero Vasar, Allen Kaasik, Anton Terasmaa, Mario Plaas
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	wolfram syndrome Wfs1 GLP1 receptor agonist liraglutide rat model neurodegeneration Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/baf6b0dceefa49f8af4e77d5cbd8b3b4
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spelling	oai:doaj.org-article:baf6b0dceefa49f8af4e77d5cbd8b3b42021-11-25T17:12:42ZEarly Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype10.3390/cells101131932073-4409https://doaj.org/article/baf6b0dceefa49f8af4e77d5cbd8b3b42021-11-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/3193https://doaj.org/toc/2073-4409Wolfram syndrome (WS), also known as a DIDMOAD (diabetes insipidus, early-onset diabetes mellitus, optic nerve atrophy and deafness) is a rare autosomal disorder caused by mutations in the Wolframin1 (<i>WFS1</i>) gene. Previous studies have revealed that glucagon-like peptide-1 receptor agonist (GLP1 RA) are effective in delaying and restoring blood glucose control in WS animal models and patients. The GLP1 RA liraglutide has also been shown to have neuroprotective properties in aged WS rats. WS is an early-onset, chronic condition. Therefore, early diagnosis and lifelong pharmacological treatment is the best solution to control disease progression. Hence, the aim of this study was to evaluate the efficacy of the long-term liraglutide treatment on the progression of WS symptoms. For this purpose, 2-month-old WS rats were treated with liraglutide up to the age of 18 months and changes in diabetes markers, visual acuity, and hearing sensitivity were monitored over the course of the treatment period. We found that treatment with liraglutide delayed the onset of diabetes and protected against vision loss in a rat model of WS. Therefore, early diagnosis and prophylactic treatment with the liraglutide may also prove to be a promising treatment option for WS patients by increasing the quality of life.Toomas JagomäeKadri SeppaRiin ReimetsMarko PastakMihkel PlaasMiriam A. HickeyKaia Grete KukkerLieve MoonsLies De GroefEero VasarAllen KaasikAnton TerasmaaMario PlaasMDPI AGarticlewolfram syndromeWfs1GLP1 receptor agonistliraglutiderat modelneurodegenerationBiology (General)QH301-705.5ENCells, Vol 10, Iss 3193, p 3193 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	wolfram syndrome Wfs1 GLP1 receptor agonist liraglutide rat model neurodegeneration Biology (General) QH301-705.5
spellingShingle	wolfram syndrome Wfs1 GLP1 receptor agonist liraglutide rat model neurodegeneration Biology (General) QH301-705.5 Toomas Jagomäe Kadri Seppa Riin Reimets Marko Pastak Mihkel Plaas Miriam A. Hickey Kaia Grete Kukker Lieve Moons Lies De Groef Eero Vasar Allen Kaasik Anton Terasmaa Mario Plaas Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype
description	Wolfram syndrome (WS), also known as a DIDMOAD (diabetes insipidus, early-onset diabetes mellitus, optic nerve atrophy and deafness) is a rare autosomal disorder caused by mutations in the Wolframin1 (<i>WFS1</i>) gene. Previous studies have revealed that glucagon-like peptide-1 receptor agonist (GLP1 RA) are effective in delaying and restoring blood glucose control in WS animal models and patients. The GLP1 RA liraglutide has also been shown to have neuroprotective properties in aged WS rats. WS is an early-onset, chronic condition. Therefore, early diagnosis and lifelong pharmacological treatment is the best solution to control disease progression. Hence, the aim of this study was to evaluate the efficacy of the long-term liraglutide treatment on the progression of WS symptoms. For this purpose, 2-month-old WS rats were treated with liraglutide up to the age of 18 months and changes in diabetes markers, visual acuity, and hearing sensitivity were monitored over the course of the treatment period. We found that treatment with liraglutide delayed the onset of diabetes and protected against vision loss in a rat model of WS. Therefore, early diagnosis and prophylactic treatment with the liraglutide may also prove to be a promising treatment option for WS patients by increasing the quality of life.
format	article
author	Toomas Jagomäe Kadri Seppa Riin Reimets Marko Pastak Mihkel Plaas Miriam A. Hickey Kaia Grete Kukker Lieve Moons Lies De Groef Eero Vasar Allen Kaasik Anton Terasmaa Mario Plaas
author_facet	Toomas Jagomäe Kadri Seppa Riin Reimets Marko Pastak Mihkel Plaas Miriam A. Hickey Kaia Grete Kukker Lieve Moons Lies De Groef Eero Vasar Allen Kaasik Anton Terasmaa Mario Plaas
author_sort	Toomas Jagomäe
title	Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype
title_short	Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype
title_full	Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype
title_fullStr	Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype
title_full_unstemmed	Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype
title_sort	early intervention and lifelong treatment with glp1 receptor agonist liraglutide in a wolfram syndrome rat model with an emphasis on visual neurodegeneration, sensorineural hearing loss and diabetic phenotype
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/baf6b0dceefa49f8af4e77d5cbd8b3b4
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Early Intervention and Lifelong Treatment with GLP1 Receptor Agonist Liraglutide in a Wolfram Syndrome Rat Model with an Emphasis on Visual Neurodegeneration, Sensorineural Hearing Loss and Diabetic Phenotype

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