The nutrient-responsive transcription factor TFE3 promotes autophagy, lysosomal biogenesis, and clearance of cellular debris JA Martina, HI Diab, L Lishu, L Jeong-A, S Patange, N Raben, ... Science signaling 7 (309), ra9-ra9, 2014 | 585 | 2014 |
Transcription factor EB (TFEB) is a new therapeutic target for Pompe disease C Spampanato, E Feeney, L Li, M Cardone, JA Lim, F Annunziata, H Zare, ... EMBO molecular medicine 5 (5), 691-706, 2013 | 351 | 2013 |
TFEB and TFE3 cooperate in the regulation of the innate immune response in activated macrophages N Pastore, OA Brady, HI Diab, JA Martina, L Sun, T Huynh, JA Lim, H Zare, ... Autophagy 12 (8), 1240-1258, 2016 | 271 | 2016 |
Pompe disease: from pathophysiology to therapy and back again JA Lim, L Li, N Raben Frontiers in aging neuroscience 6, 177, 2014 | 204 | 2014 |
Defects in calcium homeostasis and mitochondria can be reversed in Pompe disease JA Lim, L Li, O Kakhlon, R Myerowitz, N Raben Autophagy 11 (2), 385-402, 2015 | 112 | 2015 |
Modulation of mTOR signaling as a strategy for the treatment of Pompe disease JA Lim, L Li, OS Shirihai, KM Trudeau, R Puertollano, N Raben EMBO molecular medicine 9 (3), 353-370, 2017 | 85 | 2017 |
Therapeutic benefit of autophagy modulation in Pompe disease JA Lim, B Sun, R Puertollano, N Raben Molecular Therapy 26 (7), 1783-1796, 2018 | 54 | 2018 |
An emerging phenotype of central nervous system involvement in Pompe disease: from bench to bedside and beyond A Korlimarla, JA Lim, PS Kishnani, B Sun Annals of translational medicine 7 (13), 2019 | 51 | 2019 |
Intravenous injection of an AAV-PHP. B vector encoding human acid α-glucosidase rescues both muscle and CNS defects in murine Pompe disease JA Lim, H Yi, F Gao, N Raben, PS Kishnani, B Sun Molecular Therapy-Methods & Clinical Development 12, 233-245, 2019 | 48 | 2019 |
Pompe disease: Shared and unshared features of lysosomal storage disorders JA Lim, O Kakhlon, L Li, R Myerowitz, N Raben Rare Diseases 3 (1), e1068978, 2015 | 41 | 2015 |
Loss of β2-spectrin prevents cardiomyocyte differentiation and heart development JA Lim, HJ Baek, MS Jang, EK Choi, YM Lee, SJ Lee, SC Lim, JY Kim, ... Cardiovascular research 101 (1), 39-47, 2014 | 35 | 2014 |
Profiling of factor VIII mutations in Korean haemophilia A SH Hwang, MJ Kim, JA Lim, HC Kim, HS Kim Haemophilia 15 (6), 1311-1317, 2009 | 29 | 2009 |
α-Syntrophin modulates myogenin expression in differentiating myoblasts MJ Kim, SH Hwang, JA Lim, SC Froehner, ME Adams, HS Kim PLoS One 5 (12), e15355, 2010 | 26 | 2010 |
A novel gene therapy approach for GSD III using an AAV vector encoding a bacterial glycogen debranching enzyme JA Lim, SJ Choi, F Gao, PS Kishnani, B Sun Molecular Therapy Methods & Clinical Development 18, 240-249, 2020 | 15 | 2020 |
Atg5flox-derived autophagy-deficient model of Pompe disease: does it tell the whole story? JA Lim, H Zare, R Puertollano, N Raben Molecular therapy Methods & clinical development 7, 11-14, 2017 | 15 | 2017 |
Identification of a shared F8 mutation in the Korean patients with acquired hemophilia A SH Hwang, JA Lim, HC Kim, HW Lee, HS Kim The Korean Journal of Hematology 46 (1), 49, 2011 | 13 | 2011 |
Pros and cons of different ways to address dysfunctional autophagy in Pompe disease JA Lim, NK Meena, N Raben Annals of translational medicine 7 (13), 2019 | 12 | 2019 |
New insights into gastrointestinal involvement in late-onset Pompe disease: lessons learned from bench and bedside A Korlimarla, JA Lim, P McIntosh, K Zimmerman, BD Sun, PS Kishnani Journal of clinical medicine 10 (15), 3395, 2021 | 11 | 2021 |
Phosphoinositide 3-kinase/Akt signalling is responsible for the differential susceptibility of myoblasts and myotubes to menadione-induced oxidative stress JA Lim, JH Woo, HS Kim Free radical research 42 (9), 798-806, 2008 | 10 | 2008 |
α-Syntrophin is involved in the survival signaling pathway in myoblasts under menadione-induced oxidative stress JA Lim, SJ Choi, JY Moon, HS Kim Experimental Cell Research 344 (1), 1-10, 2016 | 8 | 2016 |