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Select item 110594281.

Demyelination and Na⁺ Channel Redistribution Underlie Auditory and Vestibular Dysfunction in PMP22-Null Mice

Jeong Han Lee, Seojin Park, Maria C. Perez-Flores, Yingying Chen, Mincheol Kang, Jinsil Choi, Lauren Levine, Michael Anne Gratton, Jie Zhao, Lucia Notterpek, Ebenezer N. Yamoah

eNeuro. 2024 Feb; 11(2): ENEURO.0462-23.2023. Published online 2024 Feb 9. doi: 10.1523/ENEURO.0462-23.2023

PMCID:: PMC11059428

Abstract Article PDF–10M

Select item 102084972.

In vitro myelination using explant culture of dorsal root ganglia: An efficient tool for analyzing peripheral nerve differentiation and disease modeling

Yurika Numata-Uematasu, Shuji Wakatsuki, Yuka Kobayashi-Ujiie, Kazuhisa Sakai, Noritaka Ichinohe, Toshiyuki Araki

PLoS One. 2023; 18(5): e0285897. Published online 2023 May 24. doi: 10.1371/journal.pone.0285897

PMCID:: PMC10208497

Abstract Article PDF–1.9M

Select item 97375753.

Distinct Changes in Calpain and Calpastatin during PNS Myelination and Demyelination in Rodent Models

John A. Miller, Domenica E. Drouet, Leonid M. Yermakov, Mahmoud S. Elbasiouny, Fatima Z. Bensabeur, Michael Bottomley, Keiichiro Susuki

Int J Mol Sci. 2022 Dec; 23(23): 15443. Published online 2022 Dec 6. doi: 10.3390/ijms232315443

PMCID:: PMC9737575

Abstract Article PDF–2.6M

Select item 92463924.

A translatable RNAi-driven gene therapy silences PMP22/Pmp22 genes and improves neuropathy in CMT1A mice

Marina Stavrou, Alexia Kagiava, Sarah G. Choudury, Matthew J. Jennings, Lindsay M. Wallace, Allison M. Fowler, Amanda Heslegrave, Jan Richter, Christina Tryfonos, Christina Christodoulou, Henrik Zetterberg, Rita Horvath, Scott Q. Harper, Kleopas A. Kleopa

J Clin Invest. 2022 Jul 1; 132(13): e159814. Published online 2022 Jul 1. doi: 10.1172/JCI159814

PMCID:: PMC9246392

Abstract Article PDF–3.3M

Select item 89465435.

Colocalization Analysis of Peripheral Myelin Protein-22 and Lamin-B1 in the Schwann Cell Nuclei of Wt and TrJ Mice

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

Biomolecules. 2022 Mar; 12(3): 456. Published online 2022 Mar 16. doi: 10.3390/biom12030456

PMCID:: PMC8946543

Abstract Article PDF–2.1M

Select item 83752246.

Inhibition of protein PMP22 enhances etoposide-induced cell apoptosis by p53 signaling pathway in Gastric Cancer

Jingjing Hou, Lin Wang, Jiabao Zhao, Huiqin Zhuo, Jia Cheng, Xin Chen, Wei Zheng, Zhijun Hong, Jianchun Cai

Int J Biol Sci. 2021; 17(12): 3145–3157. Published online 2021 Jul 25. doi: 10.7150/ijbs.59825

PMCID:: PMC8375224

Abstract Article PDF–2.0M

Select item 80740027.

Central Alteration in Peripheral Neuropathy of Trembler-J Mice: Hippocampal pmp22 Expression and Behavioral Profile in Anxiety Tests

Juan Pablo Damián, Lucia Vázquez Alberdi, Lucía Canclini, Gonzalo Rosso, Silvia Olivera Bravo, Mariana Martínez, Natalia Uriarte, Paul Ruiz, Miguel Calero, María Vittoria Di Tomaso, Alejandra Kun

Biomolecules. 2021 Apr; 11(4): 601. Published online 2021 Apr 19. doi: 10.3390/biom11040601

PMCID:: PMC8074002

Abstract Article PDF–2.7M

Select item 73225688.

Pmp22 super-enhancer deletion causes tomacula formation and conduction block in peripheral nerves

Harrison Pantera, Bo Hu, Daniel Moiseev, Chris Dunham, Jibraan Rashid, John J Moran, Kathleen Krentz, C Dustin Rubinstein, Seongsik Won, Jun Li, John Svaren

Hum Mol Genet. 2020 Jun 27; 29(10): 1689–1699. Published online 2020 Apr 30. doi: 10.1093/hmg/ddaa082

PMCID:: PMC7322568

Abstract Article PDF–1.1M

Select item 71456529.

Targeted PMP22 TATA-box editing by CRISPR/Cas9 reduces demyelinating neuropathy of Charcot-Marie-Tooth disease type 1A in mice

Ji-Su Lee, Jae Y Lee, Dong W Song, Hee S Bae, Hyun M Doo, Ho S Yu, Kyu J Lee, Hee K Kim, Hyun Hwang, Geon Kwak, Daesik Kim, Seokjoong Kim, Young B Hong, Jung M Lee, Byung-Ok Choi

Nucleic Acids Res. 2020 Jan 10; 48(1): 130–140. Published online 2019 Nov 12. doi: 10.1093/nar/gkz1070

PMCID:: PMC7145652

Abstract Article PDF–3.7M

Select item 695420310.

ADAR1 mediated regulation of neural crest derived melanocytes and Schwann cell development

Nadjet Gacem, Anthula Kavo, Lisa Zerad, Laurence Richard, Stephane Mathis, Raj P. Kapur, Melanie Parisot, Jeanne Amiel, Sylvie Dufour, Pierre de la Grange, Veronique Pingault, Jean Michel Vallat, Nadege Bondurand

Nat Commun. 2020; 11: 198. Published online 2020 Jan 10. doi: 10.1038/s41467-019-14090-5

PMCID:: PMC6954203

Abstract Article PDF–5.1M

Select item 679381911.

HNMP-1: A Novel Hematopoietic and Neural Membrane Protein Differentially Regulated in Neural Development and Injury

Laurel M. Bolin, Tom McNeil, Linda A. Lucian, Brigitte DeVaux, Karin Franz-Bacon, Daniel M. Gorman, Sandra Zurawski, Richard Murray, Terrill K. McClanahan

J Neurosci. 1997 Jul 15; 17(14): 5493–5502. doi: 10.1523/JNEUROSCI.17-14-05493.1997

PMCID:: PMC6793819

Abstract Article PDF–1.2M

Select item 679253112.

Overloaded Endoplasmic Reticulum–Golgi Compartments, a Possible Pathomechanism of Peripheral Neuropathies Caused by Mutations of the Peripheral Myelin Protein PMP22

Donatella D’Urso, Reinhard Prior, Regine Greiner–Petter, Anneke A. W. M. Gabreëls–Festen, Hans Werner Müller

J Neurosci. 1998 Jan 15; 18(2): 731–740. doi: 10.1523/JNEUROSCI.18-02-00731.1998

PMCID:: PMC6792531

Abstract Article PDF–764K

Select item 678254513.

Transport of Trembler-J Mutant Peripheral Myelin Protein 22 Is Blocked in the Intermediate Compartment and Affects the Transport of the Wild-Type Protein by Direct Interaction

Andreas R. Tobler, Lucia Notterpek, Roland Naef, Verdon Taylor, Ueli Suter, Eric M. Shooter

J Neurosci. 1999 Mar 15; 19(6): 2027–2036. doi: 10.1523/JNEUROSCI.19-06-02027.1999

PMCID:: PMC6782545

Abstract Article PDF–1.5M

Select item 674092714.

Emerging Role for Autophagy in the Removal of Aggresomes in Schwann Cells

Jenny Fortun, William A. Dunn, Jr, Shale Joy, Jie Li, Lucia Notterpek

J Neurosci. 2003 Nov 19; 23(33): 10672–10680. doi: 10.1523/JNEUROSCI.23-33-10672.2003

PMCID:: PMC6740927

Abstract Article PDF–676K

Select item 672600315.

Altered Ion Channels in an Animal Model of Charcot-Marie-Tooth Disease Type IA

Jérôme J. Devaux, Steven S. Scherer

J Neurosci. 2005 Feb 9; 25(6): 1470–1480. doi: 10.1523/JNEUROSCI.3328-04.2005

PMCID:: PMC6726003

Abstract Article PDF–734K

Select item 669070816.

Peripheral myelin protein 22 modulates store-operated calcium channel activity, providing insights into Charcot-Marie-Tooth disease etiology

Carlos G. Vanoye, Masayoshi Sakakura, Rose M. Follis, Alexandra J. Trevisan, Malathi Narayan, Jun Li, Charles R. Sanders, Bruce D. Carter

J Biol Chem. 2019 Aug 9; 294(32): 12054–12065. Published online 2019 Jun 18. doi: 10.1074/jbc.RA118.006248

PMCID:: PMC6690708

Abstract Article PDF–2.7M

Select item 668458817.

Follistatin-based ligand trap ACE-083 induces localized hypertrophy of skeletal muscle with functional improvement in models of neuromuscular disease

R. S. Pearsall, M. V. Davies, M. Cannell, J. Li, J. Widrick, A. W. Mulivor, S. Wallner, M. E. Troy, M. Spaits, K. Liharska, D. Sako, R. Castonguay, S. Keates, A. V. Grinberg, R. N. V. S. Suragani, R. Kumar

Sci Rep. 2019; 9: 11392. Published online 2019 Aug 6. doi: 10.1038/s41598-019-47818-w

PMCID:: PMC6684588

Abstract Article PDF–3.1M

Select item 667456618.

Peripheral Myelin Protein 22 Is in Complex with α6β4 Integrin, and Its Absence Alters the Schwann Cell Basal Lamina

Stephanie A. Amici, William A. Dunn, Jr, Andrew J. Murphy, Niels C. Adams, Nicholas W. Gale, David M. Valenzuela, George D. Yancopoulos, Lucia Notterpek

J Neurosci. 2006 Jan 25; 26(4): 1179–1189. doi: 10.1523/JNEUROSCI.2618-05.2006

PMCID:: PMC6674566

Abstract Article PDF–713K

Select item 663261319.

CNS/PNS Boundary Transgression by Central Glia in the Absence of Schwann Cells or Krox20/Egr2 Function

Fanny Coulpier, Laurence Decker, Benoît Funalot, Jean-Michel Vallat, Federico Garcia-Bragado, Patrick Charnay, Piotr Topilko

J Neurosci. 2010 Apr 28; 30(17): 5958–5967. doi: 10.1523/JNEUROSCI.0017-10.2010

PMCID:: PMC6632613

Abstract Article PDF–5.8M

Select item 660775920.

PMP22 Regulates Cholesterol Trafficking and ABCA1-Mediated Cholesterol Efflux

Ye Zhou, Joshua R. Miles, Hagai Tavori, Min Lin, Habibeh Khoshbouei, David R. Borchelt, Hannah Bazick, Gary E. Landreth, Sooyeon Lee, Sergio Fazio, Lucia Notterpek

J Neurosci. 2019 Jul 3; 39(27): 5404–5418. doi: 10.1523/JNEUROSCI.2942-18.2019