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Neurogenetics Lab

PUBLICATIONS

papers list
51

Identification and characterization of novel ERBB4 variant associated with sporadic amyotrophic lateral sclerosis (ALS)

Younghwi Kwon, Minsung Kang, Yu-Mi Jeon, Shinrye Lee, Ho-Won Lee, Jin-Sung Park, Hyung-Jun Kim [Journal of the Neurological Sciences   (2023) ]

Abstract
50

A Novel Interaction between MFN2/Marf and MARK4/PAR-1 Is Implicated in Synaptic Defects and Mitochondrial Dysfunction

Yeongmi Cheon, Sunggyu Yoon, Jae-Hyuk Lee, Kiyoung Kim, Hyung-Jun Kim, Sung Wook Hong, Ye-Rang Yun, Jiwon Shim, Sung-Hak Kim, Bingwei Lu, Mihye Lee, Seongsoo Lee [eNeuro   (2023) ]

Abstract
49

pKr-2 induces neurodegeneration via upregulation of microglial TLR4 in the hippocampus of AD brain

Sehwan Kim, Chanchal Sharma, Minsang Shin, Hyung-Jun Kim, Jaekwang Kim, Sang Ryong Kim [Brain, behavior, & immunity-health   (2023) ]

Abstract
48

Potential roles of the endoplasmic reticulum stress pathway in amyotrophic lateral sclerosis

Yu-Mi Jeon, Younghwi Kwon, Shinrye Lee, Hyung-Jun Kim [Frontiers in Aging Neuroscience   (2023) ]

Abstract
47

Long-term outcomes of edaravone in amyotrophic lateral sclerosis in South Korea: 72-week observational study

Jin-Mo Park, Donghwi Park, Hyung-Jun Kim, Jin-Sung Park [BMC neurology   (2022) ]

Abstract
46

PTK2 regulates tau-induced neurotoxicity via phosphorylation of p62 at Ser403

Shinrye Lee, Myungjin Jo, Younghwi Kwon, Yu-Mi Jeon, Seyeon Kim, Kea Joo Lee, Hyung-Jun Kim [J Neurogenet.   (2022) ]

Abstract
45

Therapeutic modulation of GSTO activity rescues FUS-associated neurotoxicity via deglutathionylation in ALS disease models

Sun Joo Cha, Seongsoo Lee, Hyun-Jun Choi, Yeo Jeong Han, Yu-Mi Jeon, Myungjin Jo, Shinrye Lee, Minyeop Nahm, Su Min Lim, Seung Hyun Kim, Hyung-Jun Kim, Kiyoung Kim [Developmental cell   (2022) ]

Abstract
44

Vitamin B12 Reduces TDP-43 Toxicity by Alleviating Oxidative Stress and Mitochondrial Dysfunction

Yu-Mi Jeon, Younghwi Kwon, Shinrye Lee, Seyeon Kim, Myungjin Jo, Seongsoo Lee, Sang Ryong Kim, Kiyoung Kim, and Hyung-Jun Kim [Antioxidants   (2021) ]

Abstract
43

HEXA-018, a Novel Inducer of Autophagy, Rescues TDP-43 Toxicity in Neuronal Cells

Shinrye Lee , Myungjin Jo , Hye Eun Lee, Yu-Mi Jeon, Seyeon Kim, Younghwi Kwon, Junghwa Woo, Shin Han, Ji Young Mun and Hyung-Jun Kim [Frontiers in Pharmacology   (2021) ]

Abstract
42

Overexpression of SIRT3 Suppresses Oxidative Stress-induced Neurotoxicity and Mitochondrial Dysfunction in Dopaminergic Neuronal Cells

Shinrye Lee, Yu-Mi Jeon, Myungjin Jo and Hyung-Jun Kim [Experimental Neurobiology   (2021) ]

Abstract
41

FUS-induced neurotoxicity is prevented by inhibiting GSK-3β in a drosophila model of amyotrophic lateral sclerosis

Hyun-Jun Choi,?Ji Young Lee,?Sun Joo Cha,?Yeo Jeong Han,?Ja Hoon Yoon,?Hyung-Jun Kim,?Kiyoung Kim [Human Molecular Genetics   (2021) ]

Abstract
40

Control of hippocampal prothrombin kringle-2 (pKr-2) expression reduces neurotoxic symptoms in five familial Alzheimer's disease mice

Sehwan Kim,?Gyeong Joon Moon,?Hyung Jun Kim,?Do-Geun Kim,?Jaekwang Kim,?Youngpyo Nam,?Chanchal Sharma,?Eunju Leem,?Shinrye Lee,?Kyu-Sung Kim,?Chang Man Ha,?Catriona McLean,?Byung Kwan Jin,?Won-Ho Shin,?Dong Woon Kim,?Yong-Seok Oh,?Chang-Won Hong,?Sang Ryong Kim [British Journal of Pharmacology   (2021) ]

Abstract
39

Toxicity of pathogenic ataxin-2 in?Drosophila?shows dependence on a pure CAG repeat sequence

Leeanne McGurk, Olivia M. Rifai, Oksana Shcherbakova, Alexandra E. Perlegos, China N. Byrns, Faith R. Carranza, Henry W. Zhou, Hyung-Jun Kim, Yongqing Zhu and Nancy M Bonini [Human Molecular Genetics   (2021) ]

Abstract
38

C9orf72-associated arginine-rich dipeptide repeats induce RNA-dependent nuclear accumulation of Staufen in neurons

Eun Seon Kim,?Chang Geon Chung,?Jeong Hyang Park,?Byung Su Ko,?Sung Soon Park,?Yoon Ha Kim,?In Jun Cha,?Jaekwang Kim,?Chang Man Ha,?Hyung-Jun Kim,?Sung Bae Lee [Human Molecular Genetics   (2021) ]

Abstract
37

Identification of Genetic Modifiers of TDP-43: Inflammatory Activation of Astrocytes for Neuroinflammation

Jae-Hong Kim,?Md Habibur Rahman,?Donghwi Park,?Myungjin Jo,?Hyung-Jun Kim,?Kyoungho Suk [Cells   (2021) ]

Abstract
36

Actin-microtubule crosslinker Pod-1 tunes PAR-1 signaling to control synaptic development and tau-mediated synaptic toxicity

Ha-Young Kang*, Hyung-Jun Kim*, Kiyoung Kim*, Seung-Il Oh, Sunggyu Yoon, Jaekwang Kim, Sangwoo Park, Yeongmi Cheon, Song Her, Mihye Lee, Bingwei Lu, Seongsoo Lee [Neurobiol Aging   (2020) ]

Abstract
35

NF-κB disinhibition contributes to dendrite defects in fly models of neurodegenerative diseases.

Myeong Hoon Han, Min Jee Kwon, Byung Su Ko, Do Young Hyeon, Davin Lee, Hyung-Jun Kim, Daehee Hwang, Sung Bae Lee [J Cell Biol.   (2020) ]

Abstract
34

The overexpression of TDP-43 in astrocytes causes neurodegeneration via a PTP1B mediated inflammatory response

Shinrye Lee*, Seyeon Kim*, Ha-Young Kang, Hye Ryeong Lim, Younghwi Kwon, Myungjin Jo, Yu-Mi Jeon, Sang Ryong Kim, Kiyoung Kim, Chang Man Ha, Seongsoo Lee#, Hyung-Jun Kim# [Journal of Neuroinflammation   (2020) ]

Abstract
33

The Role of Glial Mitochondria in α-Synuclein Toxicity

Yu-Mi Jeon, Younghwi Kwon, Myungjin Jo, Shinrye Lee, Seyeon Kim, Hyung-Jun Kim [Front Cell Dev Biol.   (2020) ]

Abstract
32

Recent Advances on the Role of GSK3β in the Pathogenesis of Amyotrophic Lateral Sclerosis

Hyun-Jun Choi, Sun Joo Cha, Jang-Won Lee, Hyung-Jun Kim, Kiyoung Kim [Brain Sci.   (2020) ]

Abstract
31

The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies

Myungjin Jo, Shinrye Lee, Yu-Mi Jeon, Seyeon Kim, Younghwi Kwon, Hyung-Jun Kim [Exp Mol Med.   (2020) ]

Abstract
30

The Role of HDAC6 in TDP-43-Induced Neurotoxicity and UPS Impairment

Shinrye Lee, Younghwi Kwon, Seyeon Kim, Myungjin Jo, Yu-Mi Jeon, Mookyung Cheon, Seongsoo Lee, Sang Ryong Kim, Kiyoung Kim, Hyung-Jun Kim [Front Cell Dev Biol.   (2020) ]

Abstract
29

Glutathione S-Transferase Rescues Motor Neuronal Toxicity in Fly Model of Amyotrophic Lateral Sclerosis

Sun Joo Cha, Yeo Jeong Han, Hyun-Jun Choi, Hyung-Jun Kim, Kiyoung Kim [Antioxidants (Basel)   (2020) ]

Abstract
28

Induction of GDNF and GFRα-1 Following AAV1-Rheb(S16H) Administration in the Hippocampus in vivo

Dongyoung Yun†, Min-Tae Jeon†, Hyung-Jun Kim†, Gyeong Joon Moon, Shinrye Lee, Chang Man Ha, Minsang Shin,6 and Sang Ryong Kim* [Exp Neurobiol   (2020) ]

Abstract
27

Behavioral evidence for geomagnetic imprinting and transgenerational inheritance in fruit flies.

In-Taek Oh, Hye-Jin Kwon, Soo-Chan Kim, Hyung-Jun Kim, Kenneth J. Lohmann, and Kwon-Seok Chae [Proc Natl Acad Sci U S A.   (2020) ]

Abstract
26

DRG2 Deficient Mice Exhibit Impaired Motor Behaviors with Reduced Striatal Dopamine Release.

Hye Ryeong Lim†, Mai-Tram Vo†, Dong Jun Kim, Unn Hwa Lee, Jong Hyuk Yoon, Hyung-Jun Kim, Jeongah Kim, Sang Ryong Kim, Jun Yeon Lee, Chae Ha Yang, Hee Young Kim, June-Seek Choi, Kijeong Kim, Esther Yang, Hyun Kim, Seongsoo Lee, Byung Ju Lee, Kyungjin Kim, Jeong Woo Park*, and Chang Man Ha* [Int J Mol Sci.   (2019) ]

Abstract
25

PTK2/FAK regulates UPS impairment via SQSTM1/p62 phosphorylation in TARDBP/TDP-43 proteinopathies

Shinrye Lee, Yu-Mi Jeon, Sun Joo Cha, Seyeon Kim, Younghwi Kwon, Myungjin Jo, You-Na Jang, Seongsoo Lee, Jaekwang Kim, Sang Ryong Kim, Kea Joo Lee, Sung Bae Lee, Kiyoung Kim*, Hyung-Jun Kim*. [Autophagy   (2019) ]

Abstract
24

Neurotrophic interactions between neurons and astrocytes following AAV1-Rheb(S16H) transduction in the hippocampus in vivo.

Min­Tae Jeon, Gyeong Joon Moon, Sehwan Kim, Minji Choi, Yong­Seok Oh, Dong Woon Kim, Hyung­Jun Kim, Kea Joo Lee, Youngshik Choe, Chang Man Ha, Il­Sung Jang, Michiko Nakamura, Catriona McLean, Won­Suk Chung, Won­Ho Shin, Seok­Geun Lee, Sang Ryong Kim [Br J Pharmacol.   (2019) ]

Abstract
23

Parkin expression reverses mitochondrial dysfunction in fused in sarcoma-induced amyotrophic lateral sclerosis.

Cha SJ, Choi HJ, Kim H-J, Choi EJ, Song KH, Im DS, Kim K [Insect Mol Biol.   (2019) ]

Abstract
22

Effects of Silibinin Against Prothrombin Kringle-2-Induced Neurotoxicity in the Nigrostriatal Dopaminergic System In Vivo.

Eunju Leem, Yong-Seok Oh, Won-Ho Shin, Byung Kwan Jin, Jae Yeong Jeong, Minsang Shin, Dong Woon Kim, Jin-Hyeok Jang, Hyung-Jun Kim, Chang Man Ha, Un Ju Jung, Gyeong Joon Moon, and Sang Ryong Kim [J Med Food   (2019) ]

Abstract
21

Genetic activation of parkin rescues TAF15-induced neurotoxicity in a Drosophila model of amyotrophic lateral sclerosis.

Youngjo Kim, Hyung-Jun Kim, Sun Joo Cha, Hyun-Jun Choi, Hayoung Kim, Sanghyun Lee, Yu-Mi Jeon, Mihye Lee, Seongsoo Lee, Kiyoung Kim [Neurobiol Aging   (2019) ]

Abstract
20

Inhibition of MEK5 suppresses TDP-43 toxicity via the mTOR-independent activation of the autophagy-lysosome pathway.

Myungjin Jo, Shinrye Lee, Kiyoung Kim, Seongsoo Lee, Sang Ryoung Kim, Hyung-Jun KIm [Biochem Biophys Res Commun.   (2019) ]

Abstract
19

Dietary Restriction and Neuroinflammation: A Potential Mechanistic Link.

Eugene Bok †, Myungjin Jo †, Shinrye Lee, Bo-Ram Lee, Jaekwang Kim * and Hyung-Jun Kim * †These authors contributed equally to this work. [Int. J. Mol. Sci.   (2019) ]

Abstract
18

Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity

Chia-Yu Chung, Amit Berson, Jason R. Kennerdell, Ashley Sartoris, Travis Unger, Silvia Porta, Hyung-Jun Kim, Edwin R. Smith, Ali Shilatifard, Vivianna Van Deerlin, Virginia M.-Y. Lee, Alice Chen-Plotkin & Nancy M. Bonini [Nat Commun.   (2018) ]

Abstract
17

Upregulation of neuronal astrocyte elevated gene-1 protects nigral dopaminergic neurons in vivo.

Eunju Leem*, Hyung-Jun Kim*, Minji Choi*, Sehwan Kim, Yong-Seok Oh, Kea Joo Lee, Young-Shik Choe, Jae-Young Um, Won-Ho Shin, Jae Yeong Jeong, Byung Kwan Jin, Dong Woon Kim, Catriona McLean, Paul B. Fisher, Nikolai Kholodilov, Kwang Seok Ahn, Jae Man Lee, Un Ju Jung, Seok-Geun Lee, Sang Ryong Kim. *These Authors equally contributed. [Cell Death&Disease   (2018) ]

Abstract
16

Protection of nigral dopaminergic neurons by AAV1 transduction with Rheb(S16H) against neurotoxic inflammation in vivo

Sehwan Kim, Gyeong Joon Moon, Yong-Seok Oh, Jungha Park, Won-Ho Shin, Jae Yeong Jeong, Kwang Shik Choi, Byung Kwan Jin, Nikolai Kholodilov, Robert E Burke, Hyung-Jun Kim, Chang Man Ha, Seok-Geun Lee, and Sang Ryong Kim [Experimental & Molecular Medicine   (2018) ]

Abstract
15

Beneficial Effects of Silibinin Against Kainic Acid-induced Neurotoxicity in the Hippocampus in vivo

Sehwan Kim, Un Ju Jung, Yong-Seok Oh, Min-Tae Jeon, Hyung-Jun Kim, Won-Ho Shin, Jungwan Hong, Sang Ryong Kim. [Experimental Neurobiology   (2017) ]

Abstract
14

Neuroprotective Effects of Protein Tyrosine Phosphatase 1B Inhibition against ER Stress-Induced Toxicity

Yu-Mi Jeon, Shinrye Lee, Seyeon Kim, Younghwi Kwon, Kiyoung Kim, Chang Geun Chung, Seongsoo Lee, Sungbae Lee, Hyung-Jun Kim [Molecules and Cells   (2017) ]

Abstract
13

Mitochondrial chaperone HSP­60 regulates anti­bacterial immunity via p38 MAP kinase signaling

Dae­Eun Jeong, Dongyeop Lee, Sun­Young Hwang, Yujin Lee, Jee­Eun Lee, Mihwa Seo, Wooseon Hwang, Keunhee Seo, Ara B Hwang, Murat Artan, Heehwa G Son, Jay­Hyun Jo, Haeshim Baek, Young Min Oh, Youngjae Ryu, Hyung­Jun Kim, Chang Man Ha, Joo­Yeon Yoo, Seung­Jae V Lee [EMBO Journal   (2017) ]

Abstract
12

Deneddylase 1 Regulates Deneddylase Activity of the Cop9 Signalosome in Drosophila Melanogaster.

Kiyoung Kim, Jeongsook Yoon, Jeongbin Yim and Hyung-Jun Kim [Insect Scinece.   (2017) ]

Abstract
11

Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis

Volodya Altanbyek, Sun-Joo Cha, Ga-Un Kang, Dai Sig Im, Seongsoo Lee, Hyung-Jun Kim, Kiyoung Kim [Biochemical and Biophysical Research Communications   (2016) ]

Abstract
10

Protective effect of Drosophila glutathione transferase omega 1 against hydrogen peroxide-induced neuronal toxicity.

So Yeon Lee a, In-Ae Lima, Ga-Un Kang, Sun-Joo Cha, Volodya Altanbyek, Hyung-Jun Kim, Seongsoo Lee, Kiyoung Kima, Jeongbin Yim [Gene.   (2015) ]

Abstract
9

Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?

Shinrye Lee and Hyung-Jun Kim [Experimental Neurobiology.   (2015) ]

Abstract
8

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.

Hyung-Jun Kim, Alya R Raphael, Eva S LaDow, Leeanne McGurk, Ross A Weber, John Q Trojanowski, Virginia M-Y Lee, Steven Finkbeiner, Aaron D Gitler & Nancy M Bonini. [Nature Genetics.   (2014) ]

Abstract
7

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis.

, Michael P. Hart, Renske Erion, Oliver D. King, Zamia Diaz, Tadashi Nakaya, Fadia Ibrahim, Hyung-Jun Kim, Jelena Mojsilovic-Petrovic, Saarene Panossian, Cecilia E. Kim, Edward C. Frackelton, Jennifer A. Solski, Kelly L. Williams, Dana Cla [Human Molecular Genetics.   (2012) ]

Abstract
6

A yeast functional screen predicts new candidate ALS disease genes.

Julien Couthouis, Michael P. Hart, James Shorter, Mariely DeJesus-Hernandez, Renske Erion, Rachel E. Oristano, Xiaoying Annie Liu, Daniel Ramos, Niti Jethava, Divya, Hosangadi, James Epstein, Ashley Chiang, Zamia Diaz, Tadashi Nakaya, Fadia Ibrahim, Hyung [PNAS   (2011) ]

Abstract
5

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.

Andrew C. Elden *, Hyung-Jun Kim*, Michael P. Hart*, Alice S. Chen-Plotkin*, Brian S. Johnson, Xiaodong Fang, Maria Armakola, Felix Geser, Robert Greene, Min Min Lu, Arun Padmanabhan, Dana Clay-Falcone, Leo McCluskey, Lauren Elman, Denise Juhr, Peter J. [Nature   (2010) ]

Abstract
4

Drosophila Cand1 regulates Cullin3-dependent E3 ligases by affecting the neddylation of Cullin3 and by controlling the stability of Cullin3 and adaptor protein.

Song-Hee Kim, Hyung-Jun Kim, Seonmi Kim and Jeongbin Yim. [Developmental Biology.   (2010) ]

Abstract
3

DEN1 deneddylates non-cullin proteins in vivo.

Yaru Chan, Jeongsook Yoon, June-Tai Wu, Hyung-Jun Kim, Kuan-Ting Pan, Jeongbin Yim and Cheng-Ting Chien. [Journal of Cell Science.   (2008) ]

Abstract
2

The F-actin/microtubule crosslinker Shot is a platform for Krasavietz-mediated translational regulation of midline axon repulsion.

Seongsoo Lee, Minyeop Nahm, Mihye Lee, Minjae Kwon, Euijae Kim, Alireza Dehghani Zadeh, Hanwei Cao, Hyung-Jun Kim, Zang Hee Lee, Seog Bae Oh, Jeongbin Yim, Peter A. Kolodziej, and Seungbok Lee. [Development.   (2007) ]

Abstract
1

Drosophila homolog of APP-BP1 (dAPP-BP1) interacts antagonistically with APPL during Drosophila development.

Hyung-Jun Kim, Song-Hee Kim, Sang-Ohk Shim, Eungsik Park, Changsoo Kim, Mark A. Tanouye, Jeongbin Yim. [Cell Death and Differentiation   (2007) ]

Abstract