Neurodegenerative Disease

Park NY, Heo Y, Yang JW, Yoo JM, Jang HJ, Jo JH, Park SJ, Lin Y, Choi J, Jeon H, Cha SJ, Bae G, Kim D, Kim J, Zeno W, Park JB, Isozumi N, Saio T, Kim SHy, Lee H, Hong BH, Nahm M, Lee YH, Hong YB (2025) Graphene quantum dots attenuate TDP-43 proteinopathy in amyotrophic lateral sclerosis. ACS Nano. 19(9):8692-8710.

대부분의 루게릭병 환자에서 TDP-43 단백질의 세포질 내 응집이 관찰되며, 이는 신경세포 기능 장애 및 사멸을 유발하는 주요 원인으로 알려져 있다. 따라서 TDP-43의 아밀로이드 피브릴과 같은 단백질 응집을 효과적으로 조절하는 것은 루게릭병 치료제 개발의 중요한 목표이다.

본 연구에서는 TDP-43 단백질이 아밀로이드 피브릴을 형성하는 구조적 규명을 통해 그래핀 양자점이 TDP-43 단백질에 직접 결합하여 응집을 억제하며 신경세포에서 액체-액체 상분리 반응을 조절하여, 신경세포에서 TDP-43 독성을 줄이고,세포 생존율을 증가시키는 효과를 확인했다. 그래핀 양자점을 루게릭병 모델 생쥐에 투여한 결과 운동 능력 저하가 지연되고, 생존율이 유의미하게 증가하는 것을 관찰했으며, 특히, 루게릭병을 유발하는 다른 원인 단백질인 FUS 및 C9orf72 돌연변이 모델에서도 그래핀 양자점이 신경 보호 효과를 보이는 것이 관찰되었다. 이는 그래핀 양자점이 루게릭병뿐만 아니라 다양한 신경퇴행성 질환 치료제 개발의 핵심 후보물질이 될 수 있음을 시사한다.

https://www.newswise.com/articles/study-adds-evidence-that-altered-fat-metabolism-enzyme-plays-key-role-in-lou-gehrig-s-disease

https://www.clinicalomics.com/topics/patient-care/neurological-disorders/study-points-to-fat-metabolism-as-drug-target-for-als/

Park NY*, Kwak G*, Doo HM*, Kim HJ, Jang SY, Lee YI, Choi BO, Hong YB. (2021) Farnesol ameliorates demyelinating phenotype in a cellular and animal model of Charcot-Marie-Tooth disease type 1A. Curr Issues Mol Biol. 43:2011-2021 *co-first author

Hong JM*, Jeon H*, Choi YC, Cho H, Hong YB#, Park HJ#.. (2021) A compound heterozygous pathogenic variant in B4GALNT1 is associated with axonal Charcot-Marie-Tooth disease. J Clin Neurol. 17(4):534-540 *co-first author #co-corresponding author

Hwang SH, Chang EH, Kwak G, Jeon H, Choi BO, Hong YB. (2021) Gait parameters as tools for analyzing phenotypic alterations of a mouse model of Charcot-Marie-Tooth disease. Anim Cell Sys. 25(1):11-18

Park HJ, Hong YB, Hong JM, Yun UK, Kim SW, Shin HY, Kim SM, Choi YC. (2021) Null variants in DYSF result in earlier symptom onset. Clin Genet. 99(3):396-406

Thenmozhi R, Lee JS, Park NY, Choi BO, Hong YB. (2020) Gene therapy options as new treatment for inherited peripheral neuropathy. Exp Neurobiol. 29(3):177-188

CMT는 유전질환임에도 불구하고, 대부분이 우성유전자변이로 발생하므로 유전자치료법 개발이 활성화되지 못함. 본 연구실에서는 다양한 방식의 우성유전자변이 제어법을 개발하고 있으며, 논문에서는 이들 방식의 현황과 특징에 대해 자세히 기술함.

관련기사: Neurology Advisor May 7, 2021

https://www.neurologyadvisor.com/topics/neuropathy/gene-therapy-a-promising-future-for-inherited-peripheral-neuropathy/

Doo HM*, Hong YB*, Han J, Moon HW, Hwang H, Kwak G, Nam SH, Kim SB, Chung KW, Kim JH, Choi BO. (2020) Short hairpin RNA treatment improves gait in a mouse model of Charcot‑Marie‑Tooth disease type 1A. Mol Med Rep. 22(6):4947-4955 *co-first author

Kim YY, Yoon JH, Um JH, Jeong DJ, Shin DJ, Hong YB, Kim JK, Kim DH, Kim C, Chung CG, Lee SB, Koh H, Yun J. (2020) PINK1 alleviates thermal hypersensitivity in a paclitaxel-induced Drosophila model of peripheral neuropathy. PLoS One 15(9):e0239126.

Kang KH, Han JE, Hong YB, Nam SH, Choi BO, Koh H. (2020) Human HSPB1 mutation recapitulates features of distal hereditary motor neuropathy (dHMN) in Drosophila. Biochem Biophys Res Commun. 521(1):220-226

Lee JS, Lee JY*, Song DW, Bae HS, Doo HM, Yu HS, Lee KJ, Kim HK, Hwang H, Kwak G, Kim D, Kim S, Hong YB*, Lee JM*, Choi BO*. (2020) Targeted PMP22 TATA-box editing by CRISPR/Cas9 reduces demyelinating neuropathy of Charcot-Marie-Tooth disease type 1A in mice. Nucleic Acids Res. 48(1):130-140 *co-corresponding author

유전질환의 치료법은 DNA 상의 유전적 변이를 직접적으로 제어하는 것이 가장 효과적으로 여겨지고 있음. 본 연구에서는 유전자가위 (Crispr/Cas9)를 이용하여 PMP22 단백질의 과발현을 줄임으로써 전세계 100만명의 환자를 보유한 CMT1A 질환에 대한 새로운 치료적 접근법을 제시하였으며, 이 연구를 토대로 새로운 치료제 개발의 가능성을 높임.

관련기사: CMT Research Foundation Nov 22, 2019

https://cmtrf.org/gene-editing-used-for-the-first-time-in-cmt/

Kim YH, Kim YH, Shin YK, Jo YR, Park DK, Song MY, Yoon BA, Nam SH, Kim JH, Choi BO, Shin HY, Kim SW, Kim SH, Hong YB, Kim JK, Park HT (2019) p75 and neural cell adhesion molecule 1 can identify pathologic Schwann cells in peripheral neuropathies. Ann Clin Transl Neurol. 6(7):1292-1301

Chang EH, Mo WM, Doo HM, Lee JS, Park HT, Choi BO, Hong YB (2019) Aminosalicylic acid reduces ER stress and Schwann cell death induced by MPZ mutations. Int J Mol Med. 44:125-134

Lee JS, Kwak G, Kim HJ, Park HT, Choi BO, Hong YB (2019) miR-381 Attenuates Peripheral Neuropathic Phenotype Caused by Overexpression of PMP22. Exp Neurobiol. 28(2): 279–288.

유전성 말초신경병증인 CMT에서 가장 빈도가 높은 CMT1A는 PMP22 유전자의 과발현으로 발생함. PMP22 유전자 발현을 제어하는 방식의 치료법 개발을 위해 PMP22 유전자 특이적인 miRNA를 발굴하고, 유전자치료법을 통해 CMT1A 마우스모델에서 유효성을 확인함.

Nam DE, Nam SH, Lee AJ, Hong YB, Choi BO, Chung KW (2018) Small Heat Shock Protein B3 (HSPB3) Mutation in an Axonal Charcot-Marie-Tooth Disease Family. J Peripher Nerv Syst. 23(1):60-66.

Park S, Choi Y, Kwak G, Hong YB, Jung N, Kim J, Choi BO, Jung SC. (2018) Application of differentiated human tonsil-derived stem cells to trembler-j mice. Muscle Nerve. 57(3):478-486.

Kim JY, Kim SH, Park JY, Koo H, Park KD, Hong YB, Chung KW, Choi BO (2017) A longitudinal clinicopathological study of two unrelated patients with Charcot-Marie-Tooth disease type 1E. Neurol India. 65(4):893-895.

Hong YB, Park JM, Yu JS, Yoo DH, Nam DE, Park HJ, Lee JS, Hwang SH, Chung KW, Choi BO (2017) Clinical characterization and genetic analysis of Korean patients with X-linked Charcot-Marie-Tooth disease type 1. J Peripher Nerv Syst. 22(3):172-181.

Lee M, Park CH, Chung HK, Kim HJ, Choi Y, Yoo JH, Yoon YC, Hong YB, Chung KW, Choi BO, Lee HW. (2017) Cerebral white matter abnormalities in patients with Charcot-Marie-Tooth disease. Ann Neurol. 81(1):147-151.

Lee JS, Chang EH, Koo OJ, Jwa DH, Mo WM, Kwak G, Moon HW, Park HT, Hong YB*, Choi BO (2017) Pmp22 mutant allele-specific siRNA alleviates demyelinating neuropathic phenotype in vivo. Neurobiol Dis. 100:99-107 *co-corresponding author

점돌연변이 (point mutation)에 의해 발생하는 유전질환의 치료법으로서 정상유전자 대신 변이유전자에만 특이적으로 작용하는 small interfering RNA (siRNA)를 발굴하고, 마우스모델을 이용하여 치료적 유효성을 규명함. 임상적으로도 적용할 수 있는 환자맞춤형 siRNA 발굴을 병행하여 향후 치료제 개발 가능성을 높임.

관련기사: 바이오스펙테이터 2016.11.25

http://www.biospectator.com/view/news_view.php?varAtcId=2235

Kim JY, Woo SY, Hong YB, Choi H, Kim J, Choi H, Mook-Jung I, Ha N, Kyung J, Jung SC, Choi BO, Koo SK. (2016) HDAC6 inhibitors rescued the defective axonal mitochondrial movement in motor neurons derived from the induced pluripotent stem cells (iPSCs) of peripheral neuropathy patients with HSPB1 mutation. Stem Cells Int. 2016:9475981

Jung N, Park S, Choi Y, Park JW, Hong YB, Park HH, Yu Y, Kwak G, Kim HS, Ryu KH, Kim JK, Jo I, Choi BO, Jung SC. (2016) Tonsil-Derived Mesenchymal Stem Cells Differentiate into a Schwann Cell Phenotype and Promote Peripheral Nerve Regeneration. Int J Mol Sci. 17(11). pii: E1867.

Hwang SH, Kim EJ, Hong YB, Joo J, Kim SM, Nam SH, Hong HD, Kim SH, Oh K, Lim JG, Cho JH, Chung KW, Choi BO (2016) Distal hereditary motor neuropathy type 7B with Dynactin 1 mutation. Mol Med Rep. 14(4):3362-8.

Lee J, Jung SC, Hong YB, Yoo JH, Koo H, Lee JH, Hong HD, Kim SB, Chung KW, Choi BO (2016) Recessive optic atrophy, sensorimotor neuropathy and cataract associated with novel compound heterozygous mutations in OPA1. Mol Med Rep. 14(1):33-40.

Kim JY, Koo H, Park KD, Choi SS, Yu JS, Hong YB, Chung KW, Choi BO. (2016) Genotype–phenotype correlation of Charcot-Marie-Tooth type 1E patients with PMP22 mutations. Genes Genom. 38(7):659-667.

Nam SH, Hong YB, Hyun YS, Nam DE, Kwak G, Hwang SH, Choi BO, Chung KW. (2016) Identification of Genetic Causes of Inherited Peripheral Neuropathies by Targeted Gene Panel Sequencing. Mol Cells. 39(5):382-8.

Hyun YS, Hong YB, Choi BO, Chung KW. (2016) Clinico-gentics in Korean Charcot-Marie-Tooth disease type 2Z with MORC2 mutations. Brain 139(Pt 7):e40.

Hong YB, Kang J, Kim JH, Lee J, Kwak G, Hyun YS, Nam SH, Hong HD, Choi YR, Jung SC, Koo H, Lee JE, Choi BO, Chung KW. (2016) DGAT2 Mutation in a Family with Autosomal Dominant Early-Onset Axonal Charcot-Marie-Tooth Disease. Hum Mutat. 37(5):473-80

Kennerson ML, Kim EJ, Siddell A, Kidambi A, Kim SM, Hong YB, Hwang SH, Chung KW, Choi BO. (2016) X-linked Charcot-Marie-Tooth disease type 6 (CMTX6) patients with a p.R158H mutation in the pyruvate dehydrogenase kinase isoenzyme 3 gene. J Peripher Nerv Syst. 2016 Mar;21(1):45-51.

Hong YB, Joo J, Hyun YS, Kwak G, Choi YR, Yeo HK, Jwa DH, Kim EJ, Mo WM, Nam SH, Kim SM, Yoo JH, Koo H, Park HT, Chung KW, Choi BO. (2016) A mutation in PMP2 causes dominant demyelinating Charcot-Marie-Tooth neuropathy. PLoS Genet. 12(2):e1005829

PMP2의 유전자에서의 변이가 demyelinating neuropathy를 일으킨다는 최초의 보고. 환자가계의 whole exome sequencing을 통하여 PMP2의 I43N 변이를 발견했으며, 마우스모델을 제작하여 표현형을 관찰함. 본 연구로 새로운 CMT의 새로운 아형이 추가되었으며 (CMT1G), NIH Online Medelian Inheritance in Man에 새로운 유전질환으로 등록됨(# 618279, https://omim.org/entry/618279).

Park HJ, Hong YB, Choi YC, Lee J, Kim EJ, L JS, Mo WM, Ki SM. Kim HI, Kim HJ, Hyun YS, Hong HD, Nam K, Jung SC, Kim SB, Kim SH, Kim DH, Oh KW, Kim SH, Yoo JH, Lee JE, Chung KW, Choi BO (2015) ADSSL1 mutation relevant to autosomal recessive adolescent-onset distal myopathy. Ann Neurol. 79(2):231-43.

Choi YR*, Hong YB*, Jung SC*, Lee JH, Kim YJ, Park HJ, Lee J, Koo H, Lee JS, Jwa DH, Jung N, Woo SY, Kim SB, Chung KW, Choi BO. (2015) A novel homozygous MPV17 mutation in two families with axonal sensorimotor polyneuropathy. BMC Neurol. 15(1):179. *co-first author

Hyun YS, Lee J, Kim HJ, Hong YB, Koo H, Smith AST, Kim DH, Choi BO, Chung KW (2015) Charcot-Marie-Tooth Disease Type 4H Resulting from Compound Heterozygous Mutations in FGD4 from Nonconsanguineous Korean Families. Ann Hum Genet. 79(6):470-479

Kim SB, Joo J, Hong YB*, Choi BO* (2015) Ultrasonography-guided transplantation facilitates perineural delivery of stem cells. Animal Cell System. 19(4):269-273 *co-corresponding author

Choi YR, Jung SC, Shin J, Yoo SY, Lee JS, Joo J, Lee J, Hong YB*, Choi BO* (2015) Development of cell models for high-throughput screening system of Charcot-Marie-Tooth disease type 1. J Genetic Medicine 12(1):25-30 *co-corresponding author

Lee J, Jung SC, Joo J, Choi YR, Moon HW, Kwak G, Yeo HK, Lee JS, Ahn HJ, Jung N, Hwang SH, Rheey J, Woo SY, Kim JY, Hong YB*, Choi BO*. (2015) Overexpression of mutant HSP27 causes axonal neuropathy in mice. Journal of Biomedical Science 22(1):43. *co-corresponding author

Park HJ, Choi YC, Kim SM, Hong YB, Kim SH, Yoon BR, Chung KW, Choi BO. (2015) Molecular Genetic Diagnosis of a Bethlem Myopathy Family with an Autosomal-Dominant COL6A1 Mutation as Evidenced by Exome Sequencing. J Clin Neurol. 11(2):183-7.

Choi YJ, Hyun YS, Nam SH, Hong YB, Koo H, Chung KW, Choi BO. (2015) Novel Compound Heterozygous Nonsense PRX Mutations in a Korean Dejerine-Sottas Neuropathy Family. J Clin Neurol. 11(1):92-96

Kim HJ, Lee J, Hong YB, Kim YJ, Lee JH, Nam SH, Choi BO, Chung KW. (2015) Ser135Phe mutation in HSPB1 (HSP27) from Charcot–Marie–Tooth disease type 2F families. Gene Genom. 37(3):295-303

Seo SD, Park HJ, Song HS, Kim HJ, Park JM, Hong YB, Chung KW, Choi BO (2014) Distal Myopathy with rimmed vacuoles confirmed by whole exome sequencing. J Life Sci. 24(3):311-317

Park MH, Woo HM, Hong YB, Park JH, Yoon BR, Park JM, Yoo JH, Koo H, Chae JH, Chung KW, Choi BO, Koo SK (2014) Recessive C10orf2 mutations in a family with infantile-onset spinocerebellar ataxia, sensorimotor polyneuropathy, and myopathy. Neurogenetics15(3):171-182

Hong YB, Lee JH, Park HJ, Choi YR, Hyun YS, Park JH, Koo H, Chung KW, Choi BO (2014) A family with axonal sensorimotor polyneuropathy with TUBB3 mutation. Mol Med Rep. 11(4):2729-2734

Hong YB, Jung SC, Lee J,Moon HS, Chung KW, Choi BO (2014) Dynamic transcriptional events in distal sural nerve revealed by transcriptome analysis. Exp Neurobiol. 23(2):169-172

Park HJ, Kim HJ, Hong YB, Nam SH, Chung KW, Choi BO (2014) A novel INF2 mutation in a Korean family with autosomal dominant intermediate Charcot-Marie-Tooth disease and focal segmental glomerulosclerosis. J Peripher Nerv Syst. 19(2):175-179

Hong YB, Jung JS, Jung SC, Chung KW, Choi BO. (2014) Application of variant calling algorithms for Mendelian disorders: lessons from whole-exome sequencing in Charcot–Marie–Tooth disease. Clin Genet. 86(3):298-299

Chung KW, Lee SS, Hong YB, Yoo JH, Choi BO. (2013) Proximal lower-limb weakness in charcot-marie-tooth disease. JAMA Neurol. 70(12):1587-1588.

Suh BC, Hong YB, Nakhro K, Nam SH, Chung KW, Choi BO (2013) Early-onset severe hereditary sensory and autonomic neuropathy type 1 with S331F SPTLC1 mutation. Mol Med Rep. 9(2):481-486

Hong YB, Lee JH, Park JM, Choi YR, Hyun YS, Yoon BR, Yoo JH, Koo H, Jung SC, Chung KW, Choi BO. (2013) A compound heterozygous mutation in HADHB gene causes an axonal Charcot-Marie-Tooth disease. BMC Med Genet. 14(1):125

Kim HJ*, Hong YB*, Park JM, Choi YR, Kim YJ, Yoon BR, Koo H, Yoo JH, Kim SB, Park M, Chung KW, Choi BO. (2013) Mutations in the PLEKHG5 gene is relevant with autosomal recessive intermediate Charcot-Marie-Tooth disease. Orphanet J Rare Dis. 8:102 *co-first author

Park J, Hyun YS, Kim YJ, Nam SH, Kim SH, Hong YB, Park JM, Chung KW, Choi BO. (2013) Exome Sequencing Reveals a Novel PRPS1 Mutation in a Family with CMTX5 without Optic Atrophy. J Clin Neurol. 9:283-288.

Park JM, Hong YB, Chung KW, Choi BO. (2013) Frequency of causative genes in Korean hereditary peripheral neuropathy patients. J Korean Assoc EMG-Electrodiagn Med. 15(1):11-19.

Park JM, Kim YJ, Yoo JH, Hong YB, Park JH, Koo H, Chung KW, Choi BO. (2013) A novel MYH7 mutation with prominent paraspinal and proximal muscle involvements. Neuromuscul Disord. 23(7):580-586.

Nakhro K, Park JM, Hong YB, Park JH, Nam SH, Yoon BR, Yoo JH, Koo H, Jung SC, Kim HR, Kim JY, Choi KG, Choi BO, Chung KW. (2013) SET binding factor 1 (SBF1) mutation causes Charcot-Marie-Tooth disease type 4B3. Neurology 81(2):165-173.

Lee SS, Lee HJ, Park JM, Hong YB, Park KD, Yoo JH, Koo H, Jung SC, Park HS, Lee JH, Lee MG, Hyun YS, Nakhro K, Chung KW, Choi BO. (2013) Proximal dominant hereditary motor and sensory neuropathy is caused by a mutation in TRK fused gene. JAMA Neurol. 70:607-615.

Choi SH, Lee DY, Chung ES, Hong YB, Kim SU, Jin BK (2005) Inhibition of thrombin-induced microglial activation and NAPDH oxidase by minocycline protects dopaminergic neurons in the substantia nigra in vivo. J Neurochem. 95: 1755-1765.

Oncology & Drug Resistance

Lee MW, Kim DS, Eom JE, Lee JW, Sung KW, Koo HH. Hong YB*, Yoo KH* (2022) Dual role of ERK2/NF-kB signaling in TRAIL sensitivity. Am J Cancer Res. 12(7):3373-3389 *co-corresponding author

Yi YW, Park NY, Park JI, Seong YS, Hong YB (2021) Doxycycline potentiates the anti-proliferation effects of gemcitabine in pancreatic cancer cells. Am J Cancer Res. 11(7):3515-3536

췌장암은 치료가 어려운 대표적인 질병으로, 현재 가장 많이 사용되는 약물인 Gemcitbabine은 약물 저항성으로 인해 치료효과가 높지 않음. 본 연구에서는 Doxycycline이 Gemcitbabine에 대한 약물 저항성을 줄일 수 있음을 보고한 논문으로 향후 췌장암 치료의 새로운 표적을 제공하였음.

Kim YY, Um JH, Shin DJ, Jeong DJ, Hong YB, Yun J. (2021) p53‐mediated regulation of mitochondrial dynamics plays a pivotal role in the senescence of various normal cells as well as cancer cells. FASEB J. 35(2):e21319

Kang HJ, Yi YW, Hong YB, Kim HJ, Jang YJ, Seong YS, Bae I (2014) HER2 confers drug resistance of human breast cancer cells through activation of NRF2 by direct interaction. Sci Rep. 4:7201

Duong HQ, Yi YW, Kang HJ, Hong YB, Tang W, Wang A, Seong YS, Bae I (2013) Inhibition of NRF2 by PIK-75 augments sensitivity of pancreatic cancer cells to gemcitabine. Int J Oncol. 44(3):959-969

Duong HQ*, Hong YB*, Kim JS, Lee HS, Yi YW, Kim YJ, Wang A, Zhao W, Cho CH, Seong YS, Bae I. (2013) Inhibition of checkpoint kinase 2 (CHK2) enhances sensitivity of pancreatic adenocarcinoma cells to gemcitabine. J Cell Mol Med. 17(10):1261-1270 *co-first author

Kang HJ*, Hong YB*, Yi YW, Cho CH, Wang A, Bae I. (2013) The correlations between BRCA1 defect and environmental factors in the risk of breast cancer. J Toxicol Sci. 38(3):355-61. *co-first author

Ramachandran S, Kwon KY, Shin SJ, Kwon SH, Cha SD, Lee HG, Hong YB, Bae I, Lee GH, Cho CH. (2013) Regulatory role of osteopontin in malignant transformation of endometrial cancer. Mol Biol Rep. 40:3623-3629.

Kang HJ, Yi YW, Kim HJ, Hong YB, Seong YS, Bae I. (2012) BRCA1 negatively regulates IGF-1 expression through an estrogen-responsive element-like site. Cell Death Dis 3:e336

Kim YJ, Hwang JS, Hong YB, Bae I, Seong YS. (2012) Transforming growth factor beta receptor I inhibitor sensitizes drug-resistant pancreatic cancer cells to gemcitabine. Anticancer Res. 32:799-806

Kang HJ, Hong YB*, Kim HJ, Wang, A, Bae I. (2012) Bioactive food components prevent carcinogenic stress via Nrf2 activation in BRCA1 deficient breast epithelial cells. Toxicol Lett. 209:154-160 *co-first author

Kim YJ, Hong YB*, Cho CH, Seong YS, Bae I. (2012) Exploring Protein Kinase Inhibitors: Unveiling Gemcitabine Resistance in Pancreatic Cancer. Pancreas 41(5):804-805 *co-first author

Hong YB, Kim JS, Yi YW, Seong YS, Bae I. (2012) Exploring Protein Kinase Inhibitors: Potentiating Gemcitabine Efficacy in Pancreatic Cancer. Pancreas 41(3):496-498

Kang HJ, Hong YB, Kim HJ, Rodriguez OC, Nath RG, Tilli EM, Albanese C, Chung FL, Kwon SH, Bae I. (2011) Detoxification: a novel function of BRCA1 in tumor suppression? Toxicological Sci. 122:26-37.

Kang HJ, Hong YB*, Kim HJ, Yi YW, Nath RG, Chang YS, Cho HC, Bae I. (2011) A novel in vitro pancreatic carcinogenesis model. Toxicology Lett. 202:15-22 *co-first author

Kang HJ, Hong YB, Kim HJ, Bae I. (2010) CR6-interacting factor 1 (Crif1) regulates NF-E2-related factor-2 (Nrf2) protein stability by proteasome-mediated degradation. J Biol Chem. 285:21258-21268.

Hong YB, Kang HJ, Kwon SY, Kim HJ, Kwon KY, Cho CH, Lee JM, Kallakury BVS, Bae I. (2010) Nrf2 regulates drug resistance in pancreatic cancer cells. Pancreas 39:463-472.

Kim YG, Ramachandran S, Kim DC, Hong YB, Kim EH, Kwon SH, Shin SJ, Cha SD, Bae I, Cho CH. (2010) Isoliquiritigenin inhibited cell proliferation and triggered apoptosis in human endometrial cancer cell line. J Women’s Med. 3:89-95.

Hong YB, Kang HJ, Kim HJ, Rosen EM, Dakshanamurthy S, Rondanin R, Baruchello R, Grisolia G, Daniele S, Bae I (2009) Inhibition of cell proliferation by a resveratrol analog in human pancreatic and breast cancer cells. Exp Mol Med. 41:151-160.

Genetic Disorders & Others

Shen JS, Edwards NJ, Hong YB, Murray GJ (2008) Isofagomine increases lysosomal delivery of exogenous glucocerebrosidase. Biochem Biophys Res Commun. 369: 1071-1075.

Hong YB, Kim EY, Jung SC (2006) Upregulation of proinflammatory cytokines in the fetal brain of the Gaucher mouse. J Korean med Sci. 21(4): 733-738.

Kim EY, Hong YB, Go SH, Lee B, Jung SC (2006) Down regulation of neurotrophic factors in the brain of a mouse model of Gaucher disease: implications for neuronal loss in Gaucher disease. Exp Mol Med. 38(4): 348-356.

Kim EY, Hong YB, Lai Z, Cho YH, Brady RO, Jung SC (2005) Long-term expression of the human glucocerebrosidase gene in vivo after transplantation of bone-marrow-derived cells transformed with a lentivirus vector. J Gene Med. 7: 878-887.

Hong YB, Kim EY, Jung SC (2004) Down regulation of Bcl-2 in the fetal brain of Gaucher disease mouse: a possible role in the neuronal loss. J Hum Genet. 49: 349-354.

Hong YB, Kim EY, Yoo HW, Jung SC (2004) Feasibility of gene therapy in Gaucher disease using an adeno-associated virus vector. J Hum Genet. 49: 536-543.

Kim EY, Hong YB, Lai Z, Kim HJ, Cho YH, Brady RO, Jung SC (2004) Expression and secretion of human glucocerebrosidase mediated by recombinant lentivirus vector in vitro and in vivo : implications for gene therapy for Gaucher disease. Biochem Biophys Res Commun. 318: 381-390.

Hong YB, Choi Y, Jung G (2004) Increased DNA polymerase fidelity of the Lamivudine resistant variants of human Hepatitis B virus DNA Polymerase. J Biochem Mol Biol. 37 (2): 167-176.

Hong YB, Kang KM, Chang NK (2001) Regional variations of cellular slime molds referred to ribosomal DNA. Korean J Ecol. 24 (6): 359-364.

Kim Y, Hong YB, Suh SW, Jung G (2000) Nucleotide insertion fidelity of human hepatitis B viral polymerase. J Biochem Mol Biol. 33 (2): 126-132.

Kim Y, Hong YB, Jung G (1999) Hepatitis B virus DNA polymerase activity of deletion mutants. Biochem Mol Biol Int. 47 (2): 301-308.

Kim Y, Hong YB, Jung G (1998) HBV polymerase residue Asp429 and Asp551, invariants at motif A and C are essential to DNA binding. J Biochem Mol Biol. 31 (5): 488-502.

Kang KM, Hong YB, Lee JB, Chang NK (1998) Occurrence and distribution of cellular slime molds in Mt. Nam. Korean J Ecol. 21 (5): 687-694.

Lee YI, Hong YB, Kim Y, Rho HM, Jung G (1997) RNase H activity of human hepatitis B virus polymerase expressed in Escherichia coli. Biochem Biophys Res Commun. 233: 401-407.

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