PROFILE

강소미 박사

Disruption of nuclear-cytoskeletal linkage by lamin A mutations in severe Emery-Dreifuss Muscular Dystrophy

EDMD-related LMNA mutations producing multi-lobular-shaped nuclei can be aberrantly aggregated, disrupt the endogenous lamina and nuclear-cytoskeleton system, and alter BIN1 localization, resulting in abnormal nuclear positioning. ASO-based therapeutic strategies can be suggested as a mutation-specific approach for correcting nuclear defects in EDMD.

부산대학교 생명시스템연구소

EDUCATION

2012.03.-2016.02. Department of Molecular Biology, Pusan National University (BS)
2016.03.-2021.08. Department of Integrated Biological Science, Pusan National University (Ph.D)

PROFESSIONAL EXPERIENCE

2021.09.-present. Institute of Systems Biology, Pusan National University (Post-doc)
2023.09.-present. Postdoctoral Fellowship Program, Next-Generation Academic Researcher Training Project, Ministry of Education, Korea

RESEARCH ACTIVITIE & OTHERS

TG Woo, J Han, M Lee, S Kang, … & BJ Park. (2025) Inhibition of SOD1 trimerization is a novel drug target for ALS disease. Translational Neurodegeneration. 12;14(1)
S Park, AY Oh, BS Hong, YJ Shin, H Jang, H Seo, S Kang, … & Park. (2024) Molecular Therapy Oncology, 10;32(4)
S Kang, S Seo, EJ Song, … & BJ Park. (2023) Progerinin, an Inhibitor of Progerin Alleviates Cardiac Abnormalities in a Model Mouse of Hutchinson-Gilford Progeria Syndrome. Cells, 12(9), 1232
S Kang, MH Yoon, SJ Lee, … & BJ Park. (2021) Human WRN is an intrinsic of progerin, abnormal splicing product of lamin A. Scientific Reports, 11, 1-14
J Ahn, J Lee, S Jeong, S Kang, BJ Park, NC Ha. (2021) Beta-strand-mediated dimeric formation of the Ig-like domain of human lamin A/C and B1. Biochemical and Biophysical Research Communications, 550, 191-196
S Kang, MH Yoon, J Ahn, … & BJ Park. (2021) Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome. Communication Biology, 4(1):5
J Ahn, TG Woo, S Kang, … & BJ Park, NC Ha. (2021) The flavonoid morin alleviates nuclear deformation in aged cells by disrupting progerin-lamin A/C binding. Journal of Functional Foods, 77

PUBLICATIONS

1. TG Woo, J Han, M Lee, S Kang, ... &BJ Park. (2025) Inhibition of SOD1 trimerization is a novel drug target for ALS
disease. Translational Neurodegeneration. 12;14(1)
2. S Park, AY Oh, BS Hong, YJ Shin, H Jang, H Seo, S Kang, ... & BJ Park. (2024) Molecular Therapy Oncology, 10;32(4)
3. S Kang, S Seo, EJ Song, ... & BJ Park. (2023) Progerinin, an Inhibitor of Progerin Alleviates Cardiac Abnormalities in
a Model Mouse of Hutchinson-Gilford Progeria Syndrome. Cells, 12(9), 1232
4. S Kang, MH Yoon, SJ Lee, ... & BJ Park. (2021) Human WRN is an intrinsic inhibitor of progerin abnormal splicing
product of lamin A. Scientific Reports, 11, 1-14
5. J Ahn, J Lee, S Jeong, S Kang, BJ Park, NC Ha. (2021) Beta-strand-mediated dimeric formation of the Ig-like domain
of human lamin A/C and B1. Biochemical and Biophysical Research Communications, 550, 191-196