Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice

Myelination of the peripheral nervous system requires Schwann cells (SC) differentiation into the myelinating phenotype. The peripheral myelin protein-22 (PMP22) is an integral membrane glycoprotein, expressed in SC. It was initially described as a growth arrest-specific (gas3) gene product, up-regu...

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Main Author: Di Tomaso, María Vittoria (author)
Other Authors: Vázquez Alberdi, Lucía (author), Olsson, Daniela (author), Cancela Bruno, Saira (author), Fernández Constenla, Anabel Sonia (author), Rosillo Martí, Juan Carlos (author), Reyes Ábalos, Ana Laura (author), Álvarez Zabaleta, Magdalena (author), Calero, Miguel (author), Kun González, Alejandra E. (author)
Format: article
Language:English
Published: 2022
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Online Access:https://hdl.handle.net/20.500.12008/38338
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_version_ 1868890175477645312
author Di Tomaso, María Vittoria
author2 Vázquez Alberdi, Lucía
Olsson, Daniela
Cancela Bruno, Saira
Fernández Constenla, Anabel Sonia
Rosillo Martí, Juan Carlos
Reyes Ábalos, Ana Laura
Álvarez Zabaleta, Magdalena
Calero, Miguel
Kun González, Alejandra E.
author2_role author
author
author
author
author
author
author
author
author
author_browse Calero, Miguel
Cancela Bruno, Saira
Di Tomaso, María Vittoria
Fernández Constenla, Anabel Sonia
Kun González, Alejandra E.
Olsson, Daniela
Reyes Ábalos, Ana Laura
Rosillo Martí, Juan Carlos
Vázquez Alberdi, Lucía
Álvarez Zabaleta, Magdalena
author_facet Di Tomaso, María Vittoria
Vázquez Alberdi, Lucía
Olsson, Daniela
Cancela Bruno, Saira
Fernández Constenla, Anabel Sonia
Rosillo Martí, Juan Carlos
Reyes Ábalos, Ana Laura
Álvarez Zabaleta, Magdalena
Calero, Miguel
Kun González, Alejandra E.
author_role author
collection COLIBRI
dc.contributor.none.fl_str_mv Di Tomaso María Vittoria, IIBCE
Vázquez Alberdi Lucía, IIBCE
Olsson Daniela, IIBCE
Cancela Bruno Saira, IIBCE
Fernández Constenla Anabel Sonia, IIBCE
Rosillo Martí Juan Carlos, IIBCE
Reyes Ábalos Ana Laura, IIBCE
Álvarez Zabaleta Magdalena, IIBCE
Calero Miguel
Kun González Alejandra E., Universidad de la República (Uruguay). Facultad de Ciencias. Instituto de Biología.
dc.creator.none.fl_str_mv Di Tomaso, María Vittoria
Vázquez Alberdi, Lucía
Olsson, Daniela
Cancela Bruno, Saira
Fernández Constenla, Anabel Sonia
Rosillo Martí, Juan Carlos
Reyes Ábalos, Ana Laura
Álvarez Zabaleta, Magdalena
Calero, Miguel
Kun González, Alejandra E.
dc.date.none.fl_str_mv 2022
2023-07-21T18:03:51Z
2023-07-21T18:03:51Z
dc.format.none.fl_str_mv 23 h.
application/pdf
dc.identifier.none.fl_str_mv Di Tomaso, M, Vázquez Alberdi, L, Olsson, D, [y otros autores]. "Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice". Biomolecules. [en línea] 2022, 12(3), 456.23 h. DOI: 10.3390/biom12030456
2218-273X
https://hdl.handle.net/20.500.12008/38338
10.3390/biom12030456
dc.language.none.fl_str_mv en
eng
dc.relation.none.fl_str_mv Biomolecules, 2022, 12(3), 456.
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
Licencia Creative Commons Atribución (CC - By 4.0)
dc.source.none.fl_str_mv reponame:COLIBRI
instname:Universidad de la República
instacron:Universidad de la República
dc.subject.none.fl_str_mv PMP22
Lamin B1
Trembler-J
Schwann cells nuclei
Colocalization-analysis
dc.title.none.fl_str_mv Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
dc.type.none.fl_str_mv Artículo
info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
description Myelination of the peripheral nervous system requires Schwann cells (SC) differentiation into the myelinating phenotype. The peripheral myelin protein-22 (PMP22) is an integral membrane glycoprotein, expressed in SC. It was initially described as a growth arrest-specific (gas3) gene product, up-regulated by serum starvation. PMP22 mutations were pathognomonic for human hereditary peripheral neuropathies, including the Charcot-Marie-Tooth disease (CMT). Trembler-J (TrJ) is a heterozygous mouse model carrying the same pmp22 point mutation as a CMT1E variant. Mutations in lamina genes have been related to a type of peripheral (CMT2B1) or central (autosomal dominant leukodystrophy) neuropathy. We explore the presence of PMP22 and Lamin B1 in Wt and TrJ SC nuclei of sciatic nerves and the colocalization of PMP22 concerning the silent heterochromatin (HC: DAPI-dark counterstaining), the transcriptionally active euchromatin (EC), and the nuclear lamina (H3K4m3 and Lamin B1 immunostaining, respectively). The results revealed that the number of TrJ SC nuclei in sciatic nerves was greater, and the SC volumes were smaller than those of Wt. The myelin protein PMP22 and Lamin B1 were detected in Wt and TrJ SC nuclei and predominantly in peripheral nuclear regions. The level of PMP22 was higher, and those of Lamin B1 lower in TrJ than in Wt mice. The level of PMP22 was higher, and those of Lamin B1 lower in TrJ than in Wt mice. PMP22 colocalized more with Lamin B1 and with the transcriptionally competent EC, than the silent HC with differences between Wt and TrJ genotypes. The results are discussed regarding the probable nuclear role of PMP22 and the relationship with TrJ neuropathy.
eu_rights_str_mv openAccess
format article
id anni_b95d4880a2a62223c79a3bbbcd149f5f
identifier_str_mv Di Tomaso, M, Vázquez Alberdi, L, Olsson, D, [y otros autores]. "Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice". Biomolecules. [en línea] 2022, 12(3), 456.23 h. DOI: 10.3390/biom12030456
2218-273X
10.3390/biom12030456
instacron_str Universidad de la República
institution Universidad de la República
instname_str Universidad de la República
language eng
language_invalid_str_mv en
network_acronym_str anni
network_name_str oai-lr-anni
oai_identifier_str oai:colibri.udelar.edu.uy:20.500.12008/38338
publishDate 2022
publishDateSort 2022
reponame_str COLIBRI
repository.mail.fl_str_mv
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repository_id_str
rights_invalid_str_mv Licencia Creative Commons Atribución (CC - By 4.0)
spelling Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ MiceDi Tomaso, María VittoriaVázquez Alberdi, LucíaOlsson, DanielaCancela Bruno, SairaFernández Constenla, Anabel SoniaRosillo Martí, Juan CarlosReyes Ábalos, Ana LauraÁlvarez Zabaleta, MagdalenaCalero, MiguelKun González, Alejandra E.PMP22Lamin B1Trembler-JSchwann cells nucleiColocalization-analysisMyelination of the peripheral nervous system requires Schwann cells (SC) differentiation into the myelinating phenotype. The peripheral myelin protein-22 (PMP22) is an integral membrane glycoprotein, expressed in SC. It was initially described as a growth arrest-specific (gas3) gene product, up-regulated by serum starvation. PMP22 mutations were pathognomonic for human hereditary peripheral neuropathies, including the Charcot-Marie-Tooth disease (CMT). Trembler-J (TrJ) is a heterozygous mouse model carrying the same pmp22 point mutation as a CMT1E variant. Mutations in lamina genes have been related to a type of peripheral (CMT2B1) or central (autosomal dominant leukodystrophy) neuropathy. We explore the presence of PMP22 and Lamin B1 in Wt and TrJ SC nuclei of sciatic nerves and the colocalization of PMP22 concerning the silent heterochromatin (HC: DAPI-dark counterstaining), the transcriptionally active euchromatin (EC), and the nuclear lamina (H3K4m3 and Lamin B1 immunostaining, respectively). The results revealed that the number of TrJ SC nuclei in sciatic nerves was greater, and the SC volumes were smaller than those of Wt. The myelin protein PMP22 and Lamin B1 were detected in Wt and TrJ SC nuclei and predominantly in peripheral nuclear regions. The level of PMP22 was higher, and those of Lamin B1 lower in TrJ than in Wt mice. The level of PMP22 was higher, and those of Lamin B1 lower in TrJ than in Wt mice. PMP22 colocalized more with Lamin B1 and with the transcriptionally competent EC, than the silent HC with differences between Wt and TrJ genotypes. The results are discussed regarding the probable nuclear role of PMP22 and the relationship with TrJ neuropathy.ANII: FCE_1_2019_1_155539Di Tomaso María Vittoria, IIBCEVázquez Alberdi Lucía, IIBCEOlsson Daniela, IIBCECancela Bruno Saira, IIBCEFernández Constenla Anabel Sonia, IIBCERosillo Martí Juan Carlos, IIBCEReyes Ábalos Ana Laura, IIBCEÁlvarez Zabaleta Magdalena, IIBCECalero MiguelKun González Alejandra E., Universidad de la República (Uruguay). Facultad de Ciencias. Instituto de Biología.2023-07-21T18:03:51Z2023-07-21T18:03:51Z2022Artículoinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion23 h.application/pdfDi Tomaso, M, Vázquez Alberdi, L, Olsson, D, [y otros autores]. "Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice". Biomolecules. [en línea] 2022, 12(3), 456.23 h. DOI: 10.3390/biom120304562218-273Xhttps://hdl.handle.net/20.500.12008/3833810.3390/biom12030456reponame:COLIBRIinstname:Universidad de la Repúblicainstacron:Universidad de la RepúblicaenengBiomolecules, 2022, 12(3), 456.Las obras depositadas en el Repositorio se rigen por la Ordenanza de los Derechos de la Propiedad Intelectual de la Universidad de la República.(Res. Nº 91 de C.D.C. de 8/III/1994 – D.O. 7/IV/1994) y por la Ordenanza del Repositorio Abierto de la Universidad de la República (Res. Nº 16 de C.D.C. de 07/10/2014)info:eu-repo/semantics/openAccessLicencia Creative Commons Atribución (CC - By 4.0)oai:colibri.udelar.edu.uy:20.500.12008/383382026-04-14T10:10:08Z
spellingShingle Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
Di Tomaso, María Vittoria
PMP22
Lamin B1
Trembler-J
Schwann cells nuclei
Colocalization-analysis
status_str publishedVersion
title Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
title_full Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
title_fullStr Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
title_full_unstemmed Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
title_short Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
title_sort Colocalization analysis of peripheral myelin protein-22 and lamin-B1 in the schwann cell nuclei of Wt and TrJ Mice
topic PMP22
Lamin B1
Trembler-J
Schwann cells nuclei
Colocalization-analysis
url https://hdl.handle.net/20.500.12008/38338