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Jeong-A Lim
Jeong-A Lim
Duke University
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Year
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
5852014
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
3512013
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
2712016
Pompe disease: from pathophysiology to therapy and back again
JA Lim, L Li, N Raben
Frontiers in aging neuroscience 6, 177, 2014
2042014
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
1122015
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
852017
Therapeutic benefit of autophagy modulation in Pompe disease
JA Lim, B Sun, R Puertollano, N Raben
Molecular Therapy 26 (7), 1783-1796, 2018
542018
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
512019
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
482019
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
412015
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
352014
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
292009
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
262010
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
152020
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
152017
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
132011
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
122019
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
112021
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
102008
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
82016
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Articles 1–20