{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T13:31:40Z","timestamp":1754487100822,"version":"3.40.4"},"reference-count":79,"publisher":"Oxford University Press (OUP)","issue":"21","license":[{"start":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T00:00:00Z","timestamp":1723507200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Center for Addiction and Mental Health"},{"name":"John B. Vincent and Juan Ausi\u00f3"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,11,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Mutations in methyl-CpG binding protein 2 (MeCP2), such as the T158M, P152R, R294X, and R306C mutations, are responsible for most Rett syndrome (RTT) cases. These mutations often result in altered protein expression that appears to correlate with changes in the nuclear size; however, the molecular details of these observations are poorly understood. Using a C2C12 cellular system expressing human MeCP2-E1 isoform as well as mouse models expressing these mutations, we show that T158M and P152R result in a decrease in MeCP2 protein, whereas R306C has a milder variation, and R294X resulted in an overall 2.5 to 3 fold increase. We also explored the potential involvement of the MeCP2 PEST domains in the proteasome-mediated regulation of MeCP2. Finally, we used the R294X mutant to gain further insight into the controversial competition between MeCP2 and histone H1 in the chromatin context. Interestingly, in R294X, MeCP2 E1 and E2 isoforms were differently affected, where the E1 isoform contributes to much of the overall protein increase observed, while E2 decreases by half. The modes of MeCP2 regulation, thus, appear to be differently regulated in the two isoforms.<\/jats:p>","DOI":"10.1093\/hmg\/ddae119","type":"journal-article","created":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T20:02:32Z","timestamp":1723579352000},"page":"1833-1845","source":"Crossref","is-referenced-by-count":1,"title":["Testing the PEST hypothesis using relevant Rett mutations in MeCP2 E1 and E2 isoforms"],"prefix":"10.1093","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0892-3745","authenticated-orcid":false,"given":"Ladan","family":"Kalani","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Microbiology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 2Y2,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bo-Hyun","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Microbiology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 2Y2,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alberto Ruiz","family":"de Chavez","sequence":"additional","affiliation":[{"name":"Department of Biology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 2Y2,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anastasia","family":"Roemer","sequence":"additional","affiliation":[{"name":"Departments of Oncology and Cell Biology , Faculty of Medicine and Dentistry, , 11560 University Ave, Edmonton, AB T6G 2H7,","place":["Canada"]},{"name":"University of Alberta , Faculty of Medicine and Dentistry, , 11560 University Ave, Edmonton, AB T6G 2H7,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Mikhailov","sequence":"additional","affiliation":[{"name":"Molecular Neuropsychiatry & Development (MiND) Lab, Campbell Family Mental Health Research Institute, Centre for Addiction and Mental Health , 250 College St, Toronto, ON M5T 1R8,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonathan K","family":"Merritt","sequence":"additional","affiliation":[{"name":"Vanderbilt Kennedy Center , Departments of Pediatrics, Pharmacology, and Special Education, , 1211 Medical Center Dr, Nashville, TN 37232,","place":["United States"]},{"name":"Vanderbilt University Medical Center and Vanderbilt University , Departments of Pediatrics, Pharmacology, and Special Education, , 1211 Medical Center Dr, Nashville, TN 37232,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katrina V","family":"Good","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Microbiology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 2Y2,","place":["Canada"]},{"name":"Molecular Neuropsychiatry & Development (MiND) Lab, Campbell Family Mental Health Research Institute, Centre for Addiction and Mental Health , 250 College St, Toronto, ON M5T 1R8,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert L","family":"Chow","sequence":"additional","affiliation":[{"name":"Department of Biology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 2Y2,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kerry R","family":"Delaney","sequence":"additional","affiliation":[{"name":"Department of Biology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 2Y2,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael J","family":"Hendzel","sequence":"additional","affiliation":[{"name":"Departments of Oncology and Cell Biology , Faculty of Medicine and Dentistry, , 11560 University Ave, Edmonton, AB T6G 2H7,","place":["Canada"]},{"name":"University of Alberta , Faculty of Medicine and Dentistry, , 11560 University Ave, Edmonton, AB T6G 2H7,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhaolan","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Genetics , Epigenetics Institute, , 3400 Civic Center Blvd, Philadelphia, PA 19104,","place":["United States"]},{"name":"University of Pennsylvania Perelman School of Medicine , Epigenetics Institute, , 3400 Civic Center Blvd, Philadelphia, PA 19104,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey L","family":"Neul","sequence":"additional","affiliation":[{"name":"Vanderbilt Kennedy Center , Departments of Pediatrics, Pharmacology, and Special Education, , 1211 Medical Center Dr, Nashville, TN 37232,","place":["United States"]},{"name":"Vanderbilt University Medical Center and Vanderbilt University , Departments of Pediatrics, Pharmacology, and Special Education, , 1211 Medical Center Dr, Nashville, TN 37232,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John B","family":"Vincent","sequence":"additional","affiliation":[{"name":"Molecular Neuropsychiatry & Development (MiND) Lab, Campbell Family Mental Health Research Institute, Centre for Addiction and Mental Health , 250 College St, Toronto, ON M5T 1R8,","place":["Canada"]},{"name":"Institute of Medical Science, University of Toronto , 27 King's College Cir, Toronto, ON M5S 1A8,","place":["Canada"]},{"name":"Department of Psychiatry, University of Toronto , 27 King College Cir, Toronto, ON M5T 1R8,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan","family":"Ausi\u00f3","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Microbiology, University of Victoria , 3800 Finnerty Rd, Victoria, BC V8W 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