Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.

Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease. The etiology and pathogenic mechanisms of the disease remain unknown, and there is no effective treatment. Here we show that intrathecal transplantation of human motor neurons derived from neural stem cells (NSC...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Hong J Lee, Kwang S Kim, Jin Ahn, Hye M Bae, Inja Lim, Seung U Kim
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2014
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/c5f75793a09a4083aafba55850da6c87
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:c5f75793a09a4083aafba55850da6c87
record_format	dspace
spelling	oai:doaj.org-article:c5f75793a09a4083aafba55850da6c872021-11-18T08:18:41ZHuman motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.1932-620310.1371/journal.pone.0097518https://doaj.org/article/c5f75793a09a4083aafba55850da6c872014-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24844281/?tool=EBIhttps://doaj.org/toc/1932-6203Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease. The etiology and pathogenic mechanisms of the disease remain unknown, and there is no effective treatment. Here we show that intrathecal transplantation of human motor neurons derived from neural stem cells (NSCs) in spinal cord of the SOD1G93A mouse ALS model delayed disease onset and extended life span of the animals. When HB1.F3.Olig2 (F3.Olig2) cells, stable immortalized human NSCs encoding the human Olig2 gene, were treated with sonic hedgehog (Shh) protein for 5-7 days, the cells expressed motor neuron cell type-specific phenotypes Hb9, Isl-1 and choline acetyltransferase (ChAT). These F3.Olig2-Shh human motor neurons were transplanted intrathecally in L5-L6 spinal cord of SOD1G93A mice, and at 4 weeks post-transplantation, transplanted F3.Olig2-Shh motor neurons expressing the neuronal phenotype markers NF, MAP2, Hb9, and ChAT were found in the ventral horn of the spinal cord. Onset of clinical signs in ALS mice with F3.Olig2-Shh motor neuron implants was delayed for 7 days and life span of animals was significantly extended by 20 days. Our results indicate that this treatment modality of intrathecal transplantation of human motor neurons derived from NSCs might be of value in the treatment of ALS patients without significant adverse effects.Hong J LeeKwang S KimJin AhnHye M BaeInja LimSeung U KimPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 9, Iss 5, p e97518 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Hong J Lee Kwang S Kim Jin Ahn Hye M Bae Inja Lim Seung U Kim Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.
description	Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease. The etiology and pathogenic mechanisms of the disease remain unknown, and there is no effective treatment. Here we show that intrathecal transplantation of human motor neurons derived from neural stem cells (NSCs) in spinal cord of the SOD1G93A mouse ALS model delayed disease onset and extended life span of the animals. When HB1.F3.Olig2 (F3.Olig2) cells, stable immortalized human NSCs encoding the human Olig2 gene, were treated with sonic hedgehog (Shh) protein for 5-7 days, the cells expressed motor neuron cell type-specific phenotypes Hb9, Isl-1 and choline acetyltransferase (ChAT). These F3.Olig2-Shh human motor neurons were transplanted intrathecally in L5-L6 spinal cord of SOD1G93A mice, and at 4 weeks post-transplantation, transplanted F3.Olig2-Shh motor neurons expressing the neuronal phenotype markers NF, MAP2, Hb9, and ChAT were found in the ventral horn of the spinal cord. Onset of clinical signs in ALS mice with F3.Olig2-Shh motor neuron implants was delayed for 7 days and life span of animals was significantly extended by 20 days. Our results indicate that this treatment modality of intrathecal transplantation of human motor neurons derived from NSCs might be of value in the treatment of ALS patients without significant adverse effects.
format	article
author	Hong J Lee Kwang S Kim Jin Ahn Hye M Bae Inja Lim Seung U Kim
author_facet	Hong J Lee Kwang S Kim Jin Ahn Hye M Bae Inja Lim Seung U Kim
author_sort	Hong J Lee
title	Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.
title_short	Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.
title_full	Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.
title_fullStr	Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.
title_full_unstemmed	Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.
title_sort	human motor neurons generated from neural stem cells delay clinical onset and prolong life in als mouse model.
publisher	Public Library of Science (PLoS)
publishDate	2014
url	https://doaj.org/article/c5f75793a09a4083aafba55850da6c87
work_keys_str_mv	AT hongjlee humanmotorneuronsgeneratedfromneuralstemcellsdelayclinicalonsetandprolonglifeinalsmousemodel AT kwangskim humanmotorneuronsgeneratedfromneuralstemcellsdelayclinicalonsetandprolonglifeinalsmousemodel AT jinahn humanmotorneuronsgeneratedfromneuralstemcellsdelayclinicalonsetandprolonglifeinalsmousemodel AT hyembae humanmotorneuronsgeneratedfromneuralstemcellsdelayclinicalonsetandprolonglifeinalsmousemodel AT injalim humanmotorneuronsgeneratedfromneuralstemcellsdelayclinicalonsetandprolonglifeinalsmousemodel AT seungukim humanmotorneuronsgeneratedfromneuralstemcellsdelayclinicalonsetandprolonglifeinalsmousemodel
_version_	1718421950263459840

Human motor neurons generated from neural stem cells delay clinical onset and prolong life in ALS mouse model.

Ejemplares similares