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Ihn Sik Seong
Observed names:
I. Seong,
I. Sik Seong,
I.S. Seong,
Ihn Sik SEONG,
Ihn Sik Seong,
Seong, Ihn Sik
show all 6
Observed institutions:
Broad Institute,
Harvard University,
Seoul National University,
Emory University,
Gwangju Institute of Science and Technology,
Massachusetts General Hospital
show all 6
ORCID:
0000-0003-4246-3356
Observed ORCID:
0000-0003-4246-3356
OpenAlex ID:
A5074068870
HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism
2005
·
Ihn Sik Seong
, Elena Ivanova
, et al.
·
Human Molecular Genetics
·
294
Crystal Structures of the HslVU Peptidase–ATPase Complex Reveal an ATP-Dependent Proteolysis Mechanism
2001
·
J. Wang
, J.J. Song
, et al.
·
Structure
·
237
PDF
Huntingtin facilitates polycomb repressive complex 2
2009
·
Ihn Sik Seong
, Juliana M. Woda
, et al.
·
Human Molecular Genetics
·
188
PDF
Nucleotide-Dependent Conformational Changes in a Protease-Associated ATPase HslU
2001
·
J. Wang
, J.J. Song
, et al.
·
Structure
·
169
PDF
Genetic modifiers of Huntington disease differentially influence motor and cognitive domains
2022
·
Jong-Min Lee
, Yuan Huang
, et al.
·
The American Journal of Human Genetics
·
115
MATR3 disruption in human and mouse associated with bicuspid aortic valve, aortic coarctation and patent ductus arteriosus
2015
·
Fabiola Quintero-Rivera
, Qiongchao J. Xi
, et al.
·
Human Molecular Genetics
·
104
PDF
Role of the GYVG Pore Motif of HslU ATPase in Protein Unfolding and Translocation for Degradation by HslV Peptidase
2005
·
Eunyong Park
, Young Min Rho
, et al.
·
Journal of Biological Chemistry
·
86
N-terminal Processing Is Essential for Release of Epithin, a Mouse Type II Membrane Serine Protease
2001
·
Eun-Gyung Cho
, Moon Gyo Kim
, et al.
·
Journal of Biological Chemistry
·
80
PDF
Translocations Disrupting PHF21A in the Potocki-Shaffer-Syndrome Region Are Associated with Intellectual Disability and Craniofacial Anomalies
2012
·
Hyung-Goo Kim
, Hyung-Goo Kim
, et al.
·
The American Journal of Human Genetics
·
77
PDF
HD CAG-correlated gene expression changes support a simple dominant gain of function
2011
·
Jessie C. Jacobsen
, Gillian C. Gregory
, et al.
·
Human Molecular Genetics
·
73
Unbiased Gene Expression Analysis Implicates the huntingtin Polyglutamine Tract in Extra-mitochondrial Energy Metabolism
2007
·
Jong-Min Lee
, Elena V Ivanova
, et al.
·
PLoS Genetics
·
73
PDF
ATP‐dependent degradation of SulA, a cell division inhibitor, by the HslVU protease in Escherichia coli
1999
·
Ihn Sik Seong
, Ji Yeon Oh
, et al.
·
FEBS Letters
·
71
Mutant Huntingtin Is Cleared from the Brain via Active Mechanisms in Huntington Disease
2020
·
Nicholas S. Caron
, Raul Banos
, et al.
·
Journal of Neuroscience
·
63
PDF
Htt CAG repeat expansion confers pleiotropic gains of mutant huntingtin function in chromatin regulation
2015
·
Marta Biagioli
, Francesco Ferrari
, et al.
·
Human Molecular Genetics
·
60
PDF
The HslU ATPase acts as a molecular chaperone in prevention of aggregation of SulA, an inhibitor of cell division in Escherichia coli
2000
·
Ihn Sik Seong
, Ji Yeon Oh
, et al.
·
FEBS Letters
·
58
PDF
The C-terminal Tails of HslU ATPase Act as a Molecular Switch for Activation of HslV Peptidase
2002
·
Ihn Sik Seong
, Min Suk Kang
, et al.
·
Journal of Biological Chemistry
·
56
PDF
Genetic modifiers of somatic expansion and clinical phenotypes in Huntington’s disease highlight shared and tissue-specific effects
2025
·
Group 1
, Jong-Min Lee
, et al.
·
Nature Genetics
·
54
PDF
Post-Translational Modifications (PTMs), Identified on Endogenous Huntingtin, Cluster within Proteolytic Domains between HEAT Repeats
2017
·
Tamara Ratovitski
, Robert N. O’Meally
, et al.
·
Journal of Proteome Research
·
51
Huntingtin's spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function
2016
·
Vijayvargia, Ravi
, Epand, Raquel F.
, et al.
·
Repository for Publications and Research Data (ETH Zurich)
·
48
PDF
ATP Binding, but Not Its Hydrolysis, Is Required for Assembly and Proteolytic Activity of the HslVU Protease inEscherichia coli
1997
·
Soon Ji Yoo
, Jae Hong Seol
, et al.
·
Biochemical and Biophysical Research Communications
·
39
Mutations causing Lopes-Maciel-Rodan syndrome are huntingtin hypomorphs
2021
·
Roy Jung
, Yejin Lee
, et al.
·
Human Molecular Genetics
·
38
The Polyglutamine Expansion at the N-Terminal of Huntingtin Protein Modulates the Dynamic Configuration and Phosphorylation of the C-Terminal HEAT Domain
2020
·
Taeyang Jung
, Baehyun Shin
, et al.
·
Structure
·
38
PDF
Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis
2011
·
Surya A. Reis
, Morgan N. Thompson
, et al.
·
Human Molecular Genetics
·
37
Mutagenesis of two N‐terminal Thr and five Ser residues in HslV, the proteolytic component of the ATP‐dependent HslVU protease
1997
·
Soon Ji Yoo
, Yoon Kyung Shim
, et al.
·
FEBS Letters
·
34
Allele-specific silencing of the gain-of-function mutation in Huntington’s disease using CRISPR/Cas9
2022
·
Jun Wan Shin
, Eun Pyo Hong
, et al.
·
JCI Insight
·
33
PDF
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Works count:
69
Citation count:
2,485
H-index:
26
I10-index:
38
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