화학공학소재연구정보센터
검색결과 : 7건
No. Article
1 Micropatterned substrates with physiological stiffness promote cell maturation and Pompe disease phenotype in human induced pluripotent stem cell-derived skeletal myocytes
Jiwlawat N, Lynch EM, Napiwocki BN, Stempien A, Ashton RS, Kamp TJ, Crone WC, Suzuki M
Biotechnology and Bioengineering, 116(9), 2377, 2019
2 Drug induced exocytosis of glycogen in Pompe disease
Turner CT, Fuller M, Hopwood JJ, Meikle PJ, Brooks DA
Biochemical and Biophysical Research Communications, 479(4), 721, 2016
3 A novel m.12908T > A mutation in the mitochondrial ND5 gene in patient with infantile-onset Pompe disease
Chamkha I, Alila-Fersi O, Mkaouar-Rebai E, Aloulou H, Kifagi C, Hachicha M, Fakhfakh F
Biochemical and Biophysical Research Communications, 429(1-2), 31, 2012
4 Proteasome inhibitors improve the function of mutant lysosomal alpha-glucosidase in fibroblasts from Pompe disease patient carrying c.546G > T mutation
Shimada Y, Nishida H, Nishiyama Y, Kobayashi H, Higuchi T, Eto Y, Ida H, Ohashi T
Biochemical and Biophysical Research Communications, 415(2), 274, 2011
5 Immortalization of murine muscle cells from lysosomal alpha-glucosidase deficient mice: A new tool to study pathophysiology and assess therapeutic strategies for Pompe disease
Douillard-Guilloux G, Mouly V, Caillaud C, Richard E
Biochemical and Biophysical Research Communications, 388(2), 333, 2009
6 Detection and imaging of non-contractile inclusions and sarcomeric anomalies in skeletal muscle by second harmonic generation combined with two-photon excited fluorescence
Ralston E, Swaim B, Czapiga M, Hwu WL, Chien YH, Pittis MG, Bembi B, Schwartz O, Plotz P, Raben N
Journal of Structural Biology, 162(3), 500, 2008
7 Hyaluronidase increases the biodistribution of acid alpha-1,4 glucosidase in the muscle of Pompe disease mice: An approach to enhance the efficacy of enzyme replacement therapy
Matalon R, Surendran S, Campbell GA, Michals-Matalon K, Tyring SK, Grady J, Cheng S, Kaye E
Biochemical and Biophysical Research Communications, 350(3), 783, 2006