Decrease in prosaposin in the Dystrophic mdx mouse brain.

<h4>Background</h4>Duchenne muscular dystrophy caused by a mutation in the X-linked dystrophin gene induces metabolic and structural disorders in the brain. A lack of dystrophin in brain structures is involved in impaired cognitive function. Prosaposin (PS), a neurotrophic factor, is abu...

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Autores principales:	Hui-Ling Gao, Cheng Li, Hiroaki Nabeka, Tetsuya Shimokawa, Naoto Kobayashi, Shouichiro Saito, Zhan-You Wang, Ya-Ming Cao, Seiji Matsuda
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/96823caf46bf4d69a2bcfe3f92f0c223
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spelling	oai:doaj.org-article:96823caf46bf4d69a2bcfe3f92f0c2232021-11-18T08:46:28ZDecrease in prosaposin in the Dystrophic mdx mouse brain.1932-620310.1371/journal.pone.0080032https://doaj.org/article/96823caf46bf4d69a2bcfe3f92f0c2232013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24244600/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Background</h4>Duchenne muscular dystrophy caused by a mutation in the X-linked dystrophin gene induces metabolic and structural disorders in the brain. A lack of dystrophin in brain structures is involved in impaired cognitive function. Prosaposin (PS), a neurotrophic factor, is abundant in the choroid plexus and various brain regions. We investigated whether PS serves as a link between dystrophin loss and gross and/or ultrastructural brain abnormalities.<h4>Methodology/principal findings</h4>The distribution of PS in the brains of juvenile and adult mdx mice was investigated by immunochemistry, Western blotting, and in situ hybridization. Immunochemistry revealed lower levels of PS in the cytoplasm of neurons of the cerebral cortex, hippocampus, cerebellum, and choroid plexus in mdx mice. Western blotting confirmed that PS levels were lower in these brain regions in both juveniles and adults. Even with low PS production in the choroids plexus, there was no significant PS decrease in cerebrospinal fluid (CSF). In situ hybridization revealed that the primary form of PS mRNA in both normal and mdx mice was Pro+9, a secretory-type PS, and the hybridization signals for Pro+9 in the above-mentioned brain regions were weaker in mdx mice than in normal mice. We also investigated mitogen-activated protein kinase signalling. Stronger activation of ERK1/2 was observed in mdx mice, ERK1/2 activity was positively correlated with PS activity, and exogenous PS18 stimulated both p-ERK1/2 and PS in SH-SY5Y cells.<h4>Conclusions/significance</h4>Low levels of PS and its receptors suggest the participation of PS in some pathological changes in the brains of mdx mice.Hui-Ling GaoCheng LiHiroaki NabekaTetsuya ShimokawaNaoto KobayashiShouichiro SaitoZhan-You WangYa-Ming CaoSeiji MatsudaPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 11, p e80032 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Hui-Ling Gao Cheng Li Hiroaki Nabeka Tetsuya Shimokawa Naoto Kobayashi Shouichiro Saito Zhan-You Wang Ya-Ming Cao Seiji Matsuda Decrease in prosaposin in the Dystrophic mdx mouse brain.
description	<h4>Background</h4>Duchenne muscular dystrophy caused by a mutation in the X-linked dystrophin gene induces metabolic and structural disorders in the brain. A lack of dystrophin in brain structures is involved in impaired cognitive function. Prosaposin (PS), a neurotrophic factor, is abundant in the choroid plexus and various brain regions. We investigated whether PS serves as a link between dystrophin loss and gross and/or ultrastructural brain abnormalities.<h4>Methodology/principal findings</h4>The distribution of PS in the brains of juvenile and adult mdx mice was investigated by immunochemistry, Western blotting, and in situ hybridization. Immunochemistry revealed lower levels of PS in the cytoplasm of neurons of the cerebral cortex, hippocampus, cerebellum, and choroid plexus in mdx mice. Western blotting confirmed that PS levels were lower in these brain regions in both juveniles and adults. Even with low PS production in the choroids plexus, there was no significant PS decrease in cerebrospinal fluid (CSF). In situ hybridization revealed that the primary form of PS mRNA in both normal and mdx mice was Pro+9, a secretory-type PS, and the hybridization signals for Pro+9 in the above-mentioned brain regions were weaker in mdx mice than in normal mice. We also investigated mitogen-activated protein kinase signalling. Stronger activation of ERK1/2 was observed in mdx mice, ERK1/2 activity was positively correlated with PS activity, and exogenous PS18 stimulated both p-ERK1/2 and PS in SH-SY5Y cells.<h4>Conclusions/significance</h4>Low levels of PS and its receptors suggest the participation of PS in some pathological changes in the brains of mdx mice.
format	article
author	Hui-Ling Gao Cheng Li Hiroaki Nabeka Tetsuya Shimokawa Naoto Kobayashi Shouichiro Saito Zhan-You Wang Ya-Ming Cao Seiji Matsuda
author_facet	Hui-Ling Gao Cheng Li Hiroaki Nabeka Tetsuya Shimokawa Naoto Kobayashi Shouichiro Saito Zhan-You Wang Ya-Ming Cao Seiji Matsuda
author_sort	Hui-Ling Gao
title	Decrease in prosaposin in the Dystrophic mdx mouse brain.
title_short	Decrease in prosaposin in the Dystrophic mdx mouse brain.
title_full	Decrease in prosaposin in the Dystrophic mdx mouse brain.
title_fullStr	Decrease in prosaposin in the Dystrophic mdx mouse brain.
title_full_unstemmed	Decrease in prosaposin in the Dystrophic mdx mouse brain.
title_sort	decrease in prosaposin in the dystrophic mdx mouse brain.
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/96823caf46bf4d69a2bcfe3f92f0c223
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Decrease in prosaposin in the Dystrophic mdx mouse brain.

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