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 (<i>gas3</i>) ge...
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MDPI AG
2022-03-01
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| Series: | Biomolecules |
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| Online Access: | https://www.mdpi.com/2218-273X/12/3/456 |
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| author | María Vittoria Di Tomaso Lucía Vázquez Alberdi Daniela Olsson Saira Cancela Anabel Fernández Juan Carlos Rosillo Ana Laura Reyes Ábalos Magdalena Álvarez Zabaleta Miguel Calero Alejandra Kun |
| author_facet | María Vittoria Di Tomaso Lucía Vázquez Alberdi Daniela Olsson Saira Cancela Anabel Fernández Juan Carlos Rosillo Ana Laura Reyes Ábalos Magdalena Álvarez Zabaleta Miguel Calero Alejandra Kun |
| author_sort | María Vittoria Di Tomaso |
| collection | DOAJ |
| 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 (<i>gas3</i>) 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 <i>pmp22</i> 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. |
| first_indexed | 2024-03-09T13:49:25Z |
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| id | doaj.art-b9880d27d4854787b6b733f10d73bcad |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-09T13:49:25Z |
| publishDate | 2022-03-01 |
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| series | Biomolecules |
| spelling | doaj.art-b9880d27d4854787b6b733f10d73bcad2023-11-30T20:53:09ZengMDPI AGBiomolecules2218-273X2022-03-0112345610.3390/biom12030456Colocalization Analysis of Peripheral Myelin Protein-22 and Lamin-B1 in the Schwann Cell Nuclei of Wt and TrJ MiceMaría Vittoria Di Tomaso0Lucía Vázquez Alberdi1Daniela Olsson2Saira Cancela3Anabel Fernández4Juan Carlos Rosillo5Ana Laura Reyes Ábalos6Magdalena Álvarez Zabaleta7Miguel Calero8Alejandra Kun9Departamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayLaboratorio de Biología Celular del Sistema Nervioso Periférico, Departamento de Proteínas y Ácidos Nucleicos, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayDepartamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayDepartamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayLaboratorio de Neurobiología Comparada, Departamento de Neurociencias Integrativas, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayLaboratorio de Neurobiología Comparada, Departamento de Neurociencias Integrativas, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayDepartamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayDepartamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayInstituto de Salud Carlos III, Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Unidad de Encefalopatías Espongiformes (UFIEC), 28029 Madrid, SpainLaboratorio de Biología Celular del Sistema Nervioso Periférico, Departamento de Proteínas y Ácidos Nucleicos, Instituto de Investigaciones Biológicas Clemente Estable, Montevideo 11600, UruguayMyelination 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 (<i>gas3</i>) 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 <i>pmp22</i> 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.https://www.mdpi.com/2218-273X/12/3/456PMP22Lamin B1Trembler-JSchwann cells nucleicolocalization-analysis |
| spellingShingle | María Vittoria Di Tomaso Lucía Vázquez Alberdi Daniela Olsson Saira Cancela Anabel Fernández Juan Carlos Rosillo Ana Laura Reyes Ábalos Magdalena Álvarez Zabaleta Miguel Calero Alejandra Kun Colocalization Analysis of Peripheral Myelin Protein-22 and Lamin-B1 in the Schwann Cell Nuclei of Wt and TrJ Mice Biomolecules PMP22 Lamin B1 Trembler-J Schwann cells nuclei colocalization-analysis |
| 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://www.mdpi.com/2218-273X/12/3/456 |
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