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Select item 112443311.

Altered GM1 catabolism affects NMDAR-mediated Ca²⁺ signaling at ER-PM junctions and increases synaptic spine formation in a GM1-gangliosidosis model

Jason A. Weesner, Ida Annunziata, Diantha van de Vlekkert, Camenzind G. Robinson, Yvan Campos, Ashutosh Mishra, Leigh E. Fremuth, Elida Gomero, Huimin Hu, Alessandra d’Azzo

Cell Rep. Author manuscript; available in PMC 2024 Jul 12.

Published in final edited form as: Cell Rep. 2024 May 28; 43(5): 114117. Published online 2024 Apr 16. doi: 10.1016/j.celrep.2024.114117

PMCID:: PMC11244331

Abstract Article PDF–3.7M

Select item 94068502.

Preclinical Enzyme Replacement Therapy with a Recombinant β-Galactosidase-Lectin Fusion for CNS Delivery and Treatment of GM1-Gangliosidosis

Jason Andrew Weesner, Ida Annunziata, Tianhong Yang, Walter Acosta, Elida Gomero, Huimin Hu, Diantha van de Vlekkert, Jorge Ayala, Xiaohui Qiu, Leigh Ellen Fremuth, David N. Radin, Carole L. Cramer, Alessandra d’Azzo

Cells. 2022 Aug; 11(16): 2579. Published online 2022 Aug 19. doi: 10.3390/cells11162579

PMCID:: PMC9406850

Abstract Article PDF–5.5M

Select item 87922733.

Dendro-somatic synaptic inputs to ganglion cells contradict receptive field and connectivity conventions in the mammalian retina

William N. Grimes, Miloslav Sedlacek, Morgan Musgrove, Amurta Nath, Hua Tian, Mrinalini Hoon, Fred Rieke, Joshua H. Singer, Jeffrey S. Diamond

Curr Biol. Author manuscript; available in PMC 2023 Jan 24.

Published in final edited form as: Curr Biol. 2022 Jan 24; 32(2): 315–328.e4. Published online 2021 Nov 24. doi: 10.1016/j.cub.2021.11.005

PMCID:: PMC8792273

Abstract Article PDF–2.4M

Select item 87744034.

Endogenous pH 6.0 β-Galactosidase Activity Is Linked to Neuronal Differentiation in the Olfactory Epithelium

José Antonio de Mera-Rodríguez, Guadalupe Álvarez-Hernán, Yolanda Gañán, Ana Santos-Almeida, Gervasio Martín-Partido, Joaquín Rodríguez-León, Javier Francisco-Morcillo

Cells. 2022 Jan; 11(2): 298. Published online 2022 Jan 16. doi: 10.3390/cells11020298

PMCID:: PMC8774403

Abstract Article PDF–5.4M

Select item 87436465.

Phenotypical changes of satellite glial cells in a murine model of G_M1‐gangliosidosis

Bei Huang, Isabel Zdora, Nicole de Buhr, Deborah Eikelberg, Wolfgang Baumgärtner, Eva Leitzen

J Cell Mol Med. 2022 Jan; 26(2): 527–539. Published online 2021 Dec 7. doi: 10.1111/jcmm.17113

PMCID:: PMC8743646

Abstract Article PDF–4.0M

Select item 83132396.

Cell-Type-Specific Gene Expression in Developing Mouse Neocortex: Intermediate Progenitors Implicated in Axon Development

Francesco Bedogni, Robert F. Hevner

Front Mol Neurosci. 2021; 14: 686034. Published online 2021 Jul 12. doi: 10.3389/fnmol.2021.686034

PMCID:: PMC8313239

Abstract Article PDF–1.6M

Select item 79387707.

BraInMap elucidates the macromolecular connectivity landscape of mammalian brain

Reza Pourhaghighi, Peter E. A. Ash, Sadhna Phanse, Florian Goebels, Lucas Z. M. Hu, Siwei Chen, Yingying Zhang, Shayne D. Wierbowski, Samantha Boudeau, Mohamed T. Moutaoufik, Ramy H. Malty, Edyta Malolepsza, Kalliopi Tsafou, Aparna Nathan, Graham Cromar, Hongbo Guo, Ali Al Abdullatif, Daniel J. Apicco, Lindsay A. Becker, Aaron D. Gitler, Stefan M. Pulst, Ahmed Youssef, Ryan Hekman, Pierre C. Havugimana, Carl A. White, Benjamin C. Blum, Antonia Ratti, Camron D. Bryant, John Parkinson, Kasper Lage, Mohan Babu, Haiyuan Yu, Gary D. Bader, Benjamin Wolozin, Andrew Emili

Cell Syst. Author manuscript; available in PMC 2021 Apr 22.

Published in final edited form as: Cell Syst. 2020 Apr 22; 10(4): 333–350.e14. doi: 10.1016/j.cels.2020.03.003

Correction in:: Cell Syst. 2020 Aug 26; 11(2): 208.

PMCID:: PMC7938770

Abstract Article PDF–2.0M

Select item 79008928.

INCREASED CELLULAR SENESCENCE IN THE MURINE AND HUMAN STENOTIC KIDNEY: EFFECT OF MESENCHYMAL STEM CELLS

Seo Rin Kim, Xiangyu Zou, Hui Tang, Amrutesh S. Puranik, Abdelrhman M. Abumoawad, Xiang-Yang Zhu, LaTonya J. Hickson, Tamara Tchkonia, Stephen C. Textor, James L. Kirkland, Lilach O. Lerman

J Cell Physiol. Author manuscript; available in PMC 2022 Feb 1.

Published in final edited form as: J Cell Physiol. 2021 Feb; 236(2): 1332–1344. Published online 2020 Jul 13. doi: 10.1002/jcp.29940

PMCID:: PMC7900892

Abstract Article PDF–2.1M

Select item 76989829.

A Single Injection of an Optimized Adeno-Associated Viral Vector into Cerebrospinal Fluid Corrects Neurological Disease in a Murine Model of GM1 Gangliosidosis

Christian Hinderer, Brenden Nosratbakhsh, Nathan Katz, James M. Wilson

Hum Gene Ther. November 2020; 31(21-22): 1169–1177. Published online 2020 Nov 13. doi: 10.1089/hum.2018.206

PMCID:: PMC7698982

Abstract Article PDF–779K

Select item 752165110.

Intracerebroventricular enzyme replacement therapy with β-galactosidase reverses brain pathologies due to GM1 gangliosidosis in mice

Joseph C. Chen, Amanda R. Luu, Nathan Wise, Rolando De Angelis, Vishal Agrawal, Linley Mangini, Jon Vincelette, Britta Handyside, Harry Sterling, Melanie J. Lo, Hio Wong, Nicole Galicia, Glenn Pacheco, Jeremy Van Vleet, Alexander Giaramita, Sylvia Fong, Sushmita M. Roy, Chuck Hague, Roger Lawrence, Sherry Bullens, Terri M. Christianson, Alessandra d'Azzo, Brett E. Crawford, Stuart Bunting, Jonathan H. LeBowitz, Gouri Yogalingam

J Biol Chem. 2020 Sep 25; 295(39): 13532–13555. Published online 2019 Sep 3. doi: 10.1074/jbc.RA119.009811

PMCID:: PMC7521651

Abstract Article PDF–7.2M

Select item 752164711.

Intermittent enzyme replacement therapy with recombinant human β-galactosidase prevents neuraminidase 1 deficiency

Amanda R. Luu, Cara Wong, Vishal Agrawal, Nathan Wise, Britta Handyside, Melanie J. Lo, Glenn Pacheco, Jessica B. Felix, Alexander Giaramita, Alessandra d'Azzo, Jon Vincelette, Sherry Bullens, Stuart Bunting, Terri M. Christianson, Charles M. Hague, Jonathan H. LeBowitz, Gouri Yogalingam

J Biol Chem. 2020 Sep 25; 295(39): 13556–13569. Published online 2020 Jul 28. doi: 10.1074/jbc.RA119.010794

Correction in:: J Biol Chem. 2020 Nov 13; 295(46): 15794.

PMCID:: PMC7521647

Abstract Article PDF–3.5M

Select item 723089912.

Axonopathy and Reduction of Membrane Resistance: Key Features in a New Murine Model of Human G_M1-Gangliosidosis

Deborah Eikelberg, Annika Lehmbecker, Graham Brogden, Witchaya Tongtako, Kerstin Hahn, Andre Habierski, Julia B. Hennermann, Hassan Y. Naim, Felix Felmy, Wolfgang Baumgärtner, Ingo Gerhauser

J Clin Med. 2020 Apr; 9(4): 1004. Published online 2020 Apr 2. doi: 10.3390/jcm9041004

PMCID:: PMC7230899

Abstract Article PDF–5.5M

Select item 593256713.

Loss of CLN7 results in depletion of soluble lysosomal proteins and impaired mTOR reactivation

Tatyana Danyukova, Khandsuren Ariunbat, Melanie Thelen, Nahal Brocke-Ahmadinejad, Sara E Mole, Stephan Storch

Hum Mol Genet. 2018 May 15; 27(10): 1711–1722. Published online 2018 Mar 5. doi: 10.1093/hmg/ddy076

PMCID:: PMC5932567

Abstract Article PDF–710K

Select item 570390514.

Stem cell senescence drives age-attenuated induction of pituitary tumours in mouse models of paediatric craniopharyngioma

Jose Mario Gonzalez-Meljem, Scott Haston, Gabriela Carreno, John R. Apps, Sara Pozzi, Christina Stache, Grace Kaushal, Alex Virasami, Leonidas Panousopoulos, Seyedeh Neda Mousavy-Gharavy, Ana Guerrero, Mamunur Rashid, Nital Jani, Colin R. Goding, Thomas S. Jacques, David J. Adams, Jesus Gil, Cynthia L. Andoniadou, Juan Pedro Martinez-Barbera

Nat Commun. 2017; 8: 1819. Published online 2017 Nov 28. doi: 10.1038/s41467-017-01992-5

PMCID:: PMC5703905

Abstract Article PDF–7.3M

Select item 561198215.

p16(Ink4a) and senescence-associated β-galactosidase can be induced in macrophages as part of a reversible response to physiological stimuli

Brandon M. Hall, Vitaly Balan, Anatoli S. Gleiberman, Evguenia Strom, Peter Krasnov, Lauren P. Virtuoso, Elena Rydkina, Slavoljub Vujcic, Karina Balan, Ilya I. Gitlin, Katerina I. Leonova, Camila R. Consiglio, Sandra O. Gollnick, Olga B. Chernova, Andrei V. Gudkov

Aging (Albany NY) 2017 Aug; 9(8): 1867–1884. Published online 2017 Aug 2. doi: 10.18632/aging.101268

PMCID:: PMC5611982

Abstract Article PDF–3.0M

Select item 534837916.

Is senescence-associated β-galactosidase a marker of neuronal senescence?

Malgorzata Piechota, Piotr Sunderland, Adrianna Wysocka, Maria Nalberczak, Malgorzata A. Sliwinska, Kasia Radwanska, Ewa Sikora

Oncotarget. 2016 Dec 6; 7(49): 81099–81109. Published online 2016 Oct 19. doi: 10.18632/oncotarget.12752

PMCID:: PMC5348379

Abstract Article PDF–12M

Select item 533836917.

Intestinal NCoR1, a regulator of epithelial cell maturation, controls neonatal hyperbilirubinemia

Shujuan Chen, Wenqi Lu, Mei-Fei Yueh, Eva Rettenmeier, Miao Liu, Miles Paszek, Johan Auwerx, Ruth T. Yu, Ronald M. Evans, Kepeng Wang, Michael Karin, Robert H. Tukey

Proc Natl Acad Sci U S A. 2017 Feb 21; 114(8): E1432–E1440. Published online 2017 Feb 6. doi: 10.1073/pnas.1700232114

Correction in:: Proc Natl Acad Sci U S A. 2017 May 16; 114(20): E4115.

PMCID:: PMC5338369

Abstract Article PDF–1.5M

Select item 533761818.

Spatiotemporal Analysis of a Glycolytic Activity Gradient Linked to Mouse Embryo Mesoderm Development

Vinay Bulusu, Nicole Prior, Marteinn T. Snaebjornsson, Andreas Kuehne, Katharina F. Sonnen, Jana Kress, Frank Stein, Carsten Schultz, Uwe Sauer, Alexander Aulehla

Dev Cell. 2017 Feb 27; 40(4): 331–341.e4. doi: 10.1016/j.devcel.2017.01.015

PMCID:: PMC5337618

Abstract Article

Select item 449239819.

Systemic AAV9 gene transfer in adult GM1 gangliosidosis mice reduces lysosomal storage in CNS and extends lifespan

Cara M. Weismann, Jennifer Ferreira, Allison M. Keeler, Qin Su, Linghua Qui, Scott A. Shaffer, Zuoshang Xu, Guangping Gao, Miguel Sena-Esteves

Hum Mol Genet. 2015 Aug 1; 24(15): 4353–4364. Published online 2015 May 10. doi: 10.1093/hmg/ddv168

PMCID:: PMC4492398

Abstract Article PDF–1002K

Select item 440927720.

Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses

Zeynep Akgoc, Miguel Sena-Esteves, Douglas R. Martin, Xianlin Han, Alessandra d’Azzo, Thomas N. Seyfried

J Lipid Res. 2015 May; 56(5): 1006–1013. doi: 10.1194/jlr.M057851

PMCID:: PMC4409277

Abstract Article PDF–558K

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