{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T04:10:51Z","timestamp":1768277451440,"version":"3.49.0"},"reference-count":85,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2022,3,16]],"date-time":"2022-03-16T00:00:00Z","timestamp":1647388800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/biom12030456","type":"journal-article","created":{"date-parts":[[2022,3,16]],"date-time":"2022-03-16T03:34:13Z","timestamp":1647401653000},"page":"456","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Colocalization Analysis of Peripheral Myelin Protein-22 and Lamin-B1 in the Schwann Cell Nuclei of Wt and TrJ Mice"],"prefix":"10.3390","volume":"12","author":[{"given":"Mar\u00eda Vittoria","family":"Di Tomaso","sequence":"first","affiliation":[{"name":"Departamento de Gen\u00e9tica, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3398-4961","authenticated-orcid":false,"given":"Luc\u00eda","family":"V\u00e1zquez Alberdi","sequence":"additional","affiliation":[{"name":"Laboratorio de Biolog\u00eda Celular del Sistema Nervioso Perif\u00e9rico, Departamento de Prote\u00ednas y \u00c1cidos Nucleicos, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2826-0931","authenticated-orcid":false,"given":"Daniela","family":"Olsson","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saira","family":"Cancela","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anabel","family":"Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Laboratorio de Neurobiolog\u00eda Comparada, Departamento de Neurociencias Integrativas, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"},{"name":"Laboratorio de Neurociencias, Facultad de Ciencias, Universidad de la Rep\u00fablica, Montevideo 11400, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan Carlos","family":"Rosillo","sequence":"additional","affiliation":[{"name":"Laboratorio de Neurobiolog\u00eda Comparada, Departamento de Neurociencias Integrativas, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"},{"name":"Departamento de Histolog\u00eda y Embriolog\u00eda, Facultad de Medicina, Universidad de la Rep\u00fablica, Montevideo 11800, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Laura","family":"Reyes \u00c1balos","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"},{"name":"Unidad de Microscop\u00eda Electr\u00f3nica de Barrido, Universidad de la Rep\u00fablica, Montevideo 11400, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1870-6979","authenticated-orcid":false,"given":"Magdalena","family":"\u00c1lvarez Zabaleta","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5366-3324","authenticated-orcid":false,"given":"Miguel","family":"Calero","sequence":"additional","affiliation":[{"name":"Instituto de Salud Carlos III, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CIBERNED), Unidad de Encefalopat\u00edas Espongiformes (UFIEC), 28029 Madrid, Spain"},{"name":"Queen Sofia Foundation Alzheimer Center, CIEN Foundation, 28031 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7070-0171","authenticated-orcid":false,"given":"Alejandra","family":"Kun","sequence":"additional","affiliation":[{"name":"Laboratorio de Biolog\u00eda Celular del Sistema Nervioso Perif\u00e9rico, Departamento de Prote\u00ednas y \u00c1cidos Nucleicos, Instituto de Investigaciones Biol\u00f3gicas Clemente Estable, Montevideo 11600, Uruguay"},{"name":"Secci\u00f3n Bioqu\u00edmica, Facultad de Ciencias, Universidad de la Rep\u00fablica, Montevideo 11400, Uruguay"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,3,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1083\/jcb.117.1.225","article-title":"Characterization of a novel peripheral nervous system myelin protein (PMP-22\/SR13)","volume":"117","author":"Snipes","year":"1992","journal-title":"J. Cell Biol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1097\/00005072-199603000-00004","article-title":"Ultrastructural distribution of PMP22 in 2. Charcot-MarieTooth disease type 1A","volume":"55","author":"Haney","year":"1996","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1016\/S0959-4388(00)00125-2","article-title":"Function of tetraspan proteins in the myelin sheath","volume":"10","author":"Bronstein","year":"2000","journal-title":"Curr. Opin. Neurobiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1851","DOI":"10.1007\/PL00012510","article-title":"Myelin sheaths: Glycoproteins involved in their formation, maintenance, and degeneration","volume":"59","author":"Quarles","year":"2002","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_5","first-page":"2924","article-title":"A growth arrest-specific (gas) gene codes for a membrane protein","volume":"10","author":"Manfioletti","year":"1990","journal-title":"Mol. Cell Biol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"3661","DOI":"10.1002\/j.1460-2075.1991.tb04933.x","article-title":"Axon regulated expression of a Schwann cell transcript that is homologous to a \u2019growth arrest-specific\u2019 gene","volume":"10","author":"Spreyer","year":"1991","journal-title":"EMBO J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"7195","DOI":"10.1073\/pnas.88.16.7195","article-title":"A myelin protein is encoded by the homologue of a growth arrest specific gene","volume":"88","author":"Welcher","year":"1991","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1016\/0005-2736(76)90050-X","article-title":"Purification and partial characterization of two glycoproteins in bovine peripheral myelin membrane","volume":"455","author":"Kitamura","year":"1976","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"7754","DOI":"10.1523\/JNEUROSCI.17-20-07754.1997","article-title":"Neurons promote the translocation of peripheral myelin protein 22 into myelin","volume":"17","author":"Pareek","year":"1997","journal-title":"J. Neurosci."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"10372","DOI":"10.1016\/S0021-9258(18)82211-6","article-title":"Detection and processing of peripheral myelin protein PMP22 in cultured Schwann cells","volume":"268","author":"Pareek","year":"1993","journal-title":"J. Biol. Chem."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1002\/1097-4547(20001001)62:1<15::AID-JNR3>3.0.CO;2-F","article-title":"Membrane topology of peripheral myelin protein 22","volume":"62","author":"Taylor","year":"2000","journal-title":"J. Neurosci. Res."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/S0079-6603(00)64003-5","article-title":"The peripheral myelin protein 22 and epithelial membrane protein family","volume":"64","author":"Jetten","year":"2000","journal-title":"Prog. Nucleic Acid. Res. Mol. Biol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"4382","DOI":"10.1073\/pnas.89.10.4382","article-title":"A leucine-to-proline mutation in the putative first transmembrane domain of the 22-kDa peripheral myelin protein in the trembler-J. mouse","volume":"89","author":"Suter","year":"1992","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1007\/s003359901015","article-title":"Regulation and expression of the murine pmp22 gene","volume":"10","author":"Kremer","year":"1999","journal-title":"Mamm. Genome"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1002\/(SICI)1096-9861(19970210)378:2<159::AID-CNE1>3.0.CO;2-2","article-title":"PMP-22 expression in the central nervous system of the embryonic mouse defines potential transverse segments and longitudinal columns","volume":"378","author":"Parmantier","year":"1997","journal-title":"J. Comp. Neurol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1080","DOI":"10.1111\/j.1460-9568.1995.tb01095.x","article-title":"Peripheral myelin protein-22 is expressed in rat and mouse brain and spinal cord motoneurons","volume":"7","author":"Parmantier","year":"1995","journal-title":"Eur. J. Neurosci."},{"key":"ref_17","first-page":"669","article-title":"Transcriptional startpoints and methylation patterns in the PMP22 promoters of peripheral nerve, leukocytes and tumor cell lines","volume":"7","author":"Huehne","year":"2001","journal-title":"Int. J. Mol. Med."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1038\/ng1195-274","article-title":"Hypermyelination and demyelinating peripheral neuropathy in Pmp22-deficient mice","volume":"11","author":"Adlkofer","year":"1995","journal-title":"Nat. Genet."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1007\/s12035-012-8370-x","article-title":"The PMP22 gene and its related diseases","volume":"47","author":"Li","year":"2013","journal-title":"Mol. Neurobiol."},{"key":"ref_20","unstructured":"Dyck, P.J., Thomas, P.K., Griffin, J.W., Low, P.A., and Poduslo, J.F. (1993). Hereditary motor and sensory neuropathies. PNP, W.B. Saunders Company."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1093\/brain\/123.7.1516","article-title":"Neurological dysfunction and axonal degeneration in Charcot\u2013Marie\u2013Tooth disease type 1A","volume":"123","author":"Krajewski","year":"2000","journal-title":"Brain"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1385\/NMM:8:1-2:23","article-title":"Neuropathology of Charcot-Marie-Tooth and related disorders","volume":"8","year":"2006","journal-title":"Neuromol. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/0092-8674(91)90613-4","article-title":"DNA duplication associated with Charcot-Marie-Tooth disease type 1A","volume":"66","author":"Lupski","year":"1991","journal-title":"Cell"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1038\/ng0692-176","article-title":"Peripheral myelin protein-22 gene maps in the duplication in chromosome 17p11.2 associated with Charcot Marie-Tooth type 1A","volume":"1","author":"Matsunami","year":"1992","journal-title":"Nat. Genet."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1038\/ng0692-171","article-title":"The peripheral myelin protein gene PMP22 is contained within the Charcot-Marie-Tooth disease type 1A duplication","volume":"1","author":"Timmerman","year":"1992","journal-title":"Nat. Genet."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/0092-8674(93)90058-X","article-title":"DNA deletion associated with hereditary neuropathy with liability to pressure palsies","volume":"72","author":"Chance","year":"1993","journal-title":"Cell"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1002\/ana.20777","article-title":"T118M PMP22 mutation causes partial loss of function and HNPP-like neuropathy","volume":"59","author":"Shy","year":"2006","journal-title":"Ann. Neurol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1038\/ng1292-288","article-title":"Identical point mutations of PMP-22 in Trembler-J mouse and Charcot-Marie-Tooth disease type 1A","volume":"2","author":"Valentijn","year":"1992","journal-title":"Nat. Genet."},{"key":"ref_29","unstructured":"Adam, M.P., Ardinger, H.H., Pagon, R.A., Wallace, S.E., Bean, L.J.H., Mirzaa, G., and Amemiya, A. (1998). Charcot-Marie-Tooth (CMT) Hereditary Neuropathy Overview, University of Washington. updated 2021."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/0022-510X(76)90138-6","article-title":"Hereditary hypertrophic neuropathy in the trembler mouse. Part 1. Histopathological studies: Light microscopy","volume":"30","author":"Low","year":"1976","journal-title":"J. Neurol. Sci."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/0022-510X(76)90139-8","article-title":"Hereditary hypertrophic neuropathy in the trembler mouse. Part 2. Histopathological studies: Electron microscopy","volume":"30","author":"Low","year":"1976","journal-title":"J. Neurol. Sci."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.jns.2019.01.030","article-title":"Rodent models with the expression of PMP22: Relevance to dysmyelinating CMT and HNPP","volume":"398","author":"Jouaud","year":"2019","journal-title":"J. Neurol. Sci."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"39","DOI":"10.3109\/01677068309107071","article-title":"The murine mutation trembler-j: Proof of semidominant expression by use of the linked vestigial tail marker","volume":"1","author":"Henry","year":"1983","journal-title":"J. Neurogenet."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1046\/j.1469-7580.1999.19530331.x","article-title":"Development of early postnatal peripheral nerve abnormalities in Trembler-J and PMP22 transgenic mice","volume":"195","author":"Robertson","year":"1999","journal-title":"J. Anat."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1002\/ana.23987","article-title":"Animal models and therapeutic prospects for Charcot-Marie-Tooth disease","volume":"74","author":"Bouhy","year":"2013","journal-title":"Ann. Neurol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1007\/BF00686066","article-title":"Development of onion bulb neuropathy in the Trembler mouse. Comparison with normal nerve maturation","volume":"32","author":"Ayers","year":"1975","journal-title":"Acta Neuropathol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1097\/00005072-198311000-00008","article-title":"Comparison of trembler and trembler-j mouse phenotypes: Varying severity of peripheral hypomyelination","volume":"42","author":"Henry","year":"1983","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1016\/j.str.2011.05.009","article-title":"Structural basis for the Trembler-J phenotype of Charcot-Marie-Tooth disease","volume":"19","author":"Sakakura","year":"2011","journal-title":"Structure"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1006\/nbdi.2000.0323","article-title":"PMP22 carrying the trembler or trembler-J mutation is intracellularly retained in myelinating Schwann cells","volume":"7","author":"Colby","year":"2000","journal-title":"Neurobiol. Dis."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/S0959-4388(96)80013-4","article-title":"Schwann cell development, differentiation and myelination","volume":"6","author":"Mirsky","year":"1996","journal-title":"Curr. Opin. Neurobiol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"a020529","DOI":"10.1101\/cshperspect.a020529","article-title":"Schwann cell myelination","volume":"7","author":"Salzer","year":"2015","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1376","DOI":"10.1002\/glia.22852","article-title":"New insights on Schwann cell development","volume":"63","author":"Monk","year":"2015","journal-title":"Glia"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1002\/j.1460-2075.1995.tb07095.x","article-title":"Retroviral-mediated gene transfer of the peripheral myelin protein PMP22 in SCs: Modulation of cell growth","volume":"14","author":"Zoidl","year":"1995","journal-title":"EMBO J."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1086\/339274","article-title":"Homozygous defects in LMNA, encoding lamin A\/C nuclear-envelope proteins, cause autosomal recessive axonal neuropathy in human (Charcot-Marie-Tooth disor- der type 2) and mouse","volume":"70","author":"Chaouch","year":"2002","journal-title":"Am. J. Hum. Genet."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1086\/302542","article-title":"A locus for an axonal form of autosomal recessive Charcot-Marie-Tooth disease maps to chromosome 1q21. 2-q21. 3","volume":"65","author":"Bouhouche","year":"1999","journal-title":"Am. J. Hum. Genet."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1007\/s10048-010-0269-y","article-title":"Genomic duplications mediate overexpression of lamin B1 in adult onset autosomal dominant leukodystrophy (ADLD) with autonomic symptoms","volume":"12","author":"Schuster","year":"2011","journal-title":"Neurogenetics"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1038\/ng1872","article-title":"Lamin B1 duplications cause autosomal dominant leukodystrophy","volume":"38","author":"Padiath","year":"2006","journal-title":"Nat. Genet."},{"key":"ref_48","unstructured":"DeLuca, R.R., Alexandre, S.R., Marques, T., Lopez de Souza, N., Merusse, J.L.B., and Neves, S.P. (1996). In Manual Para T\u00e9cnicos em Bioterismo, Winner Graph."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.jneumeth.2010.04.010","article-title":"Early phenotypical diagnoses in Trembler-J mice model","volume":"190","author":"Rosso","year":"2010","journal-title":"J. Neurosci. Methods"},{"key":"ref_50","unstructured":"(2021, March 10). AVMA Guidelines for the Euthanasia of Animals: 2020 Edition. Available online: https:\/\/www.avma.org\/sites\/default\/files\/2020-01\/2020-Euthanasia-Final-1-17-20.pdf."},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Dehghani, H. (2012). The Schwann Cell-Axon Link in Normal Condition or Neuro-Degenerative Diseases: An Immunocytochemical Approach. Applications of Immunocytochemistry, InTech.","DOI":"10.5772\/1896"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1111\/j.1365-2818.1993.tb03313.x","article-title":"Measurement of co-localization objects in dual-colour confocal images","volume":"169","author":"Manders","year":"1993","journal-title":"J. Microsc."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"jcs211847","DOI":"10.1242\/jcs.211847","article-title":"Image co-localization, co-occurrence versus correlation","volume":"131","author":"Aaron","year":"2018","journal-title":"J. Cell Sci."},{"key":"ref_54","unstructured":"Pearson, K. (1896). Mathematical Contributions to the Theovy of Evolution-III. Regression, Heredity, and Panmixia, University College."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"3993","DOI":"10.1529\/biophysj.103.038422","article-title":"Automatic and quantitative measurement of protein-protein colocalization in live cells","volume":"86","author":"Costes","year":"2004","journal-title":"Biophys. J."},{"key":"ref_56","first-page":"230","article-title":"Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids","volume":"57","author":"Kitamoto","year":"1987","journal-title":"Lab. Investig."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1002\/jmr.2176","article-title":"Myelinating and demyelinating phenotype of Trembler-J mouse (a model of 1009 Charcot-Marie-Tooth human disease) analyzed by atomic force microscopy and confocal microscopy","volume":"1010","author":"Rosso","year":"2012","journal-title":"J. Mol. Recognit."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1146\/annurev-biochem-060614-034115","article-title":"Lamins: Nuclear intermediate filament proteins with fundamental functions in nuclear mechanics and genome regulation","volume":"84","author":"Gruenbaum","year":"2015","journal-title":"Annu. Rev. Biochem."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"3626","DOI":"10.1016\/j.cub.2017.10.054","article-title":"Stall in Canonical autophagy-lysosome pathways prompts nucleophagy-based nuclear breakdown in neurodegeneration","volume":"27","author":"Baron","year":"2017","journal-title":"Curr. Biol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1083\/jcb.125.6.1201","article-title":"Dynamic properties of nuclear lamins: Lamin B is associated with sites of DNA replication","volume":"125","author":"Moir","year":"1994","journal-title":"J. Cell Biol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1671","DOI":"10.1083\/jcb.123.6.1671","article-title":"Lamin B Distribution and association with peripheral chromatin revealed by optical sectioning and electron microscopy tomography","volume":"123","author":"Belmont","year":"1993","journal-title":"J. Cell Biol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1101\/gad.223834.113","article-title":"Lamin B1 depletion in senescent cells triggers large-scale changes in gene expression and the chromatin landscape","volume":"27","author":"Shah","year":"2013","journal-title":"Genes Dev."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1016\/S0962-8924(00)01852-3","article-title":"Aggresomes, inclusion bodies and protein aggregation","volume":"10","author":"Kopito","year":"2000","journal-title":"Trend Cell Biol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.arr.2014.07.002","article-title":"Clearance of misfolded and aggregated proteins by aggrephagy and implications for aggregations diseases","volume":"16","author":"Hyttinen","year":"2014","journal-title":"Ageing Res. Rev."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1016\/j.nbd.2004.10.023","article-title":"Distinct disease mechanisms in peripheral neuropathies due to altered peripheral myelin protein 22 gene dosage or a Pmp22 point mutation","volume":"18","author":"Buchstaller","year":"2005","journal-title":"Neurobiol. Dis."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"5351","DOI":"10.1523\/JNEUROSCI.16-17-05351.1996","article-title":"Impaired differentiation of Schwann cells in transgenic mice with increased PMP22 gene dosage","volume":"16","author":"Magyar","year":"1996","journal-title":"J. Neurosci."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1046\/j.1469-7580.1997.19030423.x","article-title":"Abnormal Schwann cell\/axon interactions in the Trembler-J mouse","volume":"190","author":"Robertson","year":"1997","journal-title":"J. Anat."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1046\/j.1469-7580.2002.00039.x","article-title":"Comparison of a new pmp22 transgenic mouse line with other mouse models and human patients with CMT1A","volume":"200","author":"Robertson","year":"2002","journal-title":"J. Anat."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1002\/jnr.490400202","article-title":"Peripheral Myelin Protein 22: Facts and Hypotheses","volume":"40","author":"Suter","year":"1995","journal-title":"J. Neurosci. Res."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1002\/jnr.490370412","article-title":"Differential expression of two mRNA species indicates a dual function of peripheral myelin protein PMP22 in cell growth and myelination","volume":"37","author":"Bosse","year":"1994","journal-title":"J. Neurosci. Res."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1097\/00001622-199801000-00013","article-title":"The role of beta-catenin in cell adhesion, signal transduction and cancer","volume":"10","author":"Bullions","year":"1998","journal-title":"Curr. Opin. Oncol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1038\/nrm.2015.3","article-title":"Beyond \u03b2-catenin: Prospects for a larger catenin network in the nucleus","volume":"17","author":"McCrea","year":"2016","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/j.devcel.2016.07.022","article-title":"Keratins are going nuclear","volume":"38","author":"Hobbs","year":"2016","journal-title":"Dev. Cell"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1038\/nature06405","article-title":"The molecular architecture of the nuclear pore complex","volume":"450","author":"Alber","year":"2007","journal-title":"Nature"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1146\/annurev-biochem-060109-151030","article-title":"The structure of the nuclear pore complex","volume":"80","author":"Hoelz","year":"2011","journal-title":"Annu. Rev. Biochem."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1320","DOI":"10.1093\/emboj\/20.6.1320","article-title":"Kinetic analysis of translocation through nuclear pore complexes","volume":"20","author":"Ribbeck","year":"2001","journal-title":"EMBO J."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1083\/jcb.201601004","article-title":"Simple rules for passive diffusion through the nuclear pore complex","volume":"215","author":"Timney","year":"2016","journal-title":"J. Cell Biol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"2697","DOI":"10.1038\/emboj.2009.225","article-title":"Cargo surface hydrophobicity is sufficient to overcome the nuclear pore complex selectivity barrier","volume":"28","author":"Naim","year":"2009","journal-title":"EMBO J."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/j.cell.2018.05.045","article-title":"Surface properties determining passage rates of proteins through nuclear pores","volume":"174","author":"Frey","year":"2018","journal-title":"Cell"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"5101","DOI":"10.1074\/jbc.R600026200","article-title":"Classical nuclear localization signals: Definition, function, and interaction with importin alpha","volume":"282","author":"Lange","year":"2007","journal-title":"J. Biol. Chem."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1042\/BJ20150368","article-title":"Nuclear localization signals for four distinct karyopherin-\u03b2 nuclear import systems","volume":"468","author":"Soniat","year":"2015","journal-title":"Biochem. J."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0960-9822(98)70082-X","article-title":"Nuclear localization signaling dependent and importin\/karyopherin-independent nuclear import of beta-catenin","volume":"8","author":"Fagotto","year":"1998","journal-title":"Curr. Biol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1016\/j.yexcr.2016.09.009","article-title":"Distinct hydrophobic \u201cpatches\u201d in the N- and Ctails of beta-catenin contribute to nuclear transport","volume":"348","author":"Sharma","year":"2016","journal-title":"Exp. Cell. Res."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"33839","DOI":"10.1074\/jbc.M302078200","article-title":"SOX9 defect of calmodulin-dependent nuclear import in campomelic dysplasia\/autosomal sex reversal","volume":"278","author":"Argentaro","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1007\/978-1-59745-466-7_6","article-title":"Nuclear import properties of the sex determining factor SRY","volume":"390","author":"Forwood","year":"2007","journal-title":"Methods Mol. Biol."}],"container-title":["Biomolecules"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2218-273X\/12\/3\/456\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:37:15Z","timestamp":1760135835000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2218-273X\/12\/3\/456"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,16]]},"references-count":85,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,3]]}},"alternative-id":["biom12030456"],"URL":"https:\/\/doi.org\/10.3390\/biom12030456","relation":{},"ISSN":["2218-273X"],"issn-type":[{"value":"2218-273X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,16]]}}}