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114. Ectopic Lymphoid Structures Harbor Desmoglein-Specific B Cells in the Chronic Skin Lesions of Patients with Pemphigus

Author

Kim, Soo-Chan

Abstract

Pemphigus is a chronic autoimmune bullous diseases characterized by the production of autoantibodies against desmogleins (desmoglein 1 and 3). Antibody-producing B cells are usually developed and activated in germinal centers of secondary lymphoid organs such as lymph nodes and spleen. Ectopic lymphoid structures (ELSs) resembling the germinal centers have been recognized at inflamed tissues of various infectious or autoimmune diseases; however, the ELSs remain undetermined in the skin lesions of autoimmune bullous diseases including pemphigus. Here, we found tight clusters of B cells and CD4+ T cells in the dermis of chronic skin lesions from 6 patients with pemphigus vulgaris, 3 patients with pemphigus foliaceus, and 1 patient with paraneoplastic pemphigus. The clusters in the dermis contain peripheral node addressin+ venules which are only observed in secondary lymphoid organs. Furthermore, lymphotoxin u03b2, podoplanin+ venules, follicular dendritic cells, and CD11c+ dendritic cells were also detected in the ELSs. Desmoglein-specific B cells and plasma cells are present inner and peripheral areas of ELSs, whereas CD4+ T cells do not show the features of T follicular helper cells (e.g. PD-1, bcl-6) in CXCL13-enriched ELSs. In summary, we identified desmoglein-specific B cells in the dermal ELSs of chronic skin lesions from the patients with pemphigus.

Published
2017

115. DSG3 Deficiency in a Patient with Suprabasal Acantholytic Blisters in Oral and Laryngeal Mucosa

Author

Kim, Soo-Chan

Abstract

DSG3 Deficiency in a Patient with Suprabasal Acantholytic Blisters in Oral and Laryngeal MucosaJong Hoon Kim1, Song-Ee Kim1, Hae Seok Park1, Si-Hyung Lee1, Sang Eun Lee1, Soo-Chan Kim1*1Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.kimsc@yuhs.acIntroduction : Desmoglein (DSG) 3, encoded by DSG3 gene, is the major component of desmosomes which contribute to cell-cell adhesion in epidermis. Development of autoimmunity against DSG3 causes mucosal type of pemphigus vulgaris which is characterized by suprabasal acantholytic blisters limited to the mucosa, however, mutations in DSG3 gene to cause human disorder have not been found so far. Objective : Here, we report a novel DSG3 mutation as a cause of inherited blistering disease affecting oral mucosa.Methods : A 1 year-old female baby presented with recurrent erosions in the oral and laryngeal mucosa since birth. Whole genome sequencing (WGS) was performed using the blood sample of the patient. Mutation and restriction fragment length polymorphisms (RFLP) analysis was conducted with the genomic DNA of her and her family. Enzyme-linked immunosorbent assay (ELISA) was examined with her serum. H&E staining, immunofluorescence (IF) staining, and electron microscopic examination were analyzed using her skin and mucosal tissues. Immunoblotting was performed using primary keratinocyte from her skin and mucosa.Results : Her conjunctival and genital mucosa is spared and her hair was normally growing. Histological examination of skin biopsy showed suprabasal acantholytic blisters. Direct and indirect IF and ELISA for DSG1 and DSG3 were negative. WGS from her DNA identified a homozygous nonsense c.859C>T DSG3 mutation which is expected to result in premature termination of protein translation in the third extracellular cadherin domain of DSG3 (p.R287*). Direct sequencing and RFLP analysis of affected family revealed that the mutation is hereditary. In the IF studies using the skin and oral mucosa and immunoblotting studies using primary cultured keratinocytes, loss of DSG3 was detected in the epidermis of the skin and oral mucosa. Conclusion : We describe a new hereditary disease caused by an autosomal-recessive homozygous nonsense mutation in DSG3.

Published
2017

117. Peptide nucleic acid (PNA) probe-based analysis to detect filaggrin mutations in atopic dermatitis patients

Author

Hwang, Joonsung, primary, Lee, Sangku, additional, Kim, Daehwan, additional, Han, Goeun, additional, Soung, Nak Kyun, additional, Cha-Molstad, Hyunjoo, additional, Lee, Kyung Ho, additional, Ryoo, In Ja, additional, Ahn, Mi Ja, additional, Kim, Sung Tae, additional, Lee, Min Jae, additional, Yoo, Young Dong, additional, Lee, Hee Gu, additional, Hong, Jin Tae, additional, Kim, Hyunjung, additional, Choi, Eung Ho, additional, Kim, Soo-Chan, additional, Kwon, Yong Tae, additional, Ahn, Jong Seog, additional, and Kim, Bo Yeon, additional

Published
2018
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136. Programmed cell death-ligand 1 ameliorates psoriatic inflammation by inhibiting TCR-induced IL-17A production of T cells overexpressing PD-1

Author

Kim, Jong Hoon, primary, Choi, Young Joon, additional, Lee, Byung Ha, additional, Song, Mi-Young, additional, Ban, Chae Yeon, additional, Kim, Jihye, additional, Park, Junsik, additional, Kim, Song-Ee, additional, Kim, Tae-Gyun, additional, Park, Su-Hyung, additional, Kim, Hyoung-Pyo, additional, Sung, Young-Chul, additional, Kim, Soo-Chan, additional, and Shin, Eui-Cheol, additional

Published
2016
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137. Clinical Features and Awareness of Hand Eczema in Korea

Author

Park, Jae Beom, primary, Lee, Seung Ho, additional, Kim, Kea Jeung, additional, Lee, Ga-Young, additional, Yang, Jun-Mo, additional, Kim, Do Won, additional, Lee, Seok Jong, additional, Lee, Cheol Heon, additional, Park, Eun Joo, additional, Kim, Kyu Han, additional, Eun, Hee Chul, additional, Chang, Sung Eun, additional, Moon, Kee Chan, additional, Kim, Seong Hyun, additional, Kim, Seong Jin, additional, Kim, Byung-Soo, additional, Lee, Jun Young, additional, Kim, Hyung-Ok, additional, Kang, Hoon, additional, Lee, Min Geol, additional, Kim, Soo-Chan, additional, Ro, Young Suck, additional, Ko, Joo Yeon, additional, Park, Mi Youn, additional, Kim, Myung Hwa, additional, Shin, Jeong Hyun, additional, Choi, Hae Young, additional, Hong, Chang Kwun, additional, Lee, Sung Yul, additional, Bak, Hana, additional, Son, Sang Wook, additional, and Lee, Ai Young, additional

Published
2016
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144. Autoantibodies to Multiple Epitopes on the Non-Collagenous-1 Domain of Type VII Collagen Induce Blisters

Author

Vorobyev, Artem, primary, Ujiie, Hideyuki, additional, Recke, Andreas, additional, Buijsrogge, Jacqueline J.A., additional, Jonkman, Marcel F., additional, Pas, Hendri H., additional, Iwata, Hiroaki, additional, Hashimoto, Takashi, additional, Kim, Soo-Chan, additional, Hoon Kim, Jong, additional, Groves, Richard, additional, Samavedam, Unni, additional, Gupta, Yask, additional, Schmidt, Enno, additional, Zillikens, Detlef, additional, Shimizu, Hiroshi, additional, and Ludwig, Ralf J., additional

Published
2015
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148. A quantitative analysis of gait patterns in vestibular neuritis patients using gyroscope sensor and a continuous walking protocol

Author

Kim, Soo Chan, Kim, Joo, Lee, Hwan, Kwon, Jae, Kim, Nam beom, Kim, Mi, Hwang, Jong, Han, Gyu, Kim, Soo Chan, Kim, Joo, Lee, Hwan, Kwon, Jae, Kim, Nam beom, Kim, Mi, Hwang, Jong, and Han, Gyu

Abstract

Background Locomotion involves an integration of vision, proprioception, and vestibular information. The parieto-insular vestibular cortex is known to affect the supra-spinal rhythm generators, and the vestibular system regulates anti- gravity muscle tone of the lower leg in the same side to maintain an upright posture through the extra-pyramidal track. To demonstrate the relationship between locomotion and vestibular function, we evaluated the differences in gait patterns between vestibular neuritis (VN) patients and normal subjects using a gyroscope sensor and long-way walking protocol. Methods Gyroscope sensors were attached to both shanks of healthy controls ( n =10) and age-matched VN patients ( n = 10). We then asked the participants to walk 88.8 m along a corridor. Through the summation of gait cycle data, we measured gait frequency (Hz), normalized angular velocity (NAV) of each axis for legs, maximum and minimum NAV, up-slope and down-slope of NAV in swing phase, stride-swing-stance time (s), and stance to stride ratio (%). Results The most dominant walking frequency in the VN group was not different compared to normal control. The NAVs of z -axis (pitch motion) were significantly larger than the others ( x -, y -axis) and the values in VN patients tended to decrease in both legs and the difference of NAV between both group was significant in the ipsi- lesion side in the VN group only ( p =0.03). Additionally, the gait velocity of these individuals was decreased relatively to controls (1.11 ± 0.120 and 0.84 ± 0.061 m/s in control and VN group respectively, p <0.01), which seems to be related to the significantly increased stance and stride time of the ipsi- lesion side. Moreover, in the VN group, the maximum NAV of the lesion side was less, and the minimum one was higher than control group. Furthermore, the down-slope and up-slope of NAV decreased on the impaired side. Conclusion The walking pattern of VN patients was highly phase-dependent, and NA

Published
2014

149. The characteristic comparison of the accelerometer and the gyroscope in the measurement of human body sway

Author

Kaewkannate, Kanitthika, Han, Gyu Cheol, Kim, Sung Min, Kim, Soo Chan, Kaewkannate, Kanitthika, Han, Gyu Cheol, Kim, Sung Min, and Kim, Soo Chan

Abstract

This study investigated the human body sway measuring instruments. An accelerometer and a gyroscope were used to examine patients with postural control related diseases in many studies. Some studies used either an accelerometer or a gyroscope attached to the head, chest, or waist to obtain the balance assessment parameters of body sway such as area, direction, etc. The purpose of this study is to identify the reliability between both sensors in human body sway analysis by assuming the human body sway as a simple pendulum model, and suggest an optimal measurement method using the acceleration and the gyroscope. The characteristic differences between the accelerometer and the gyroscope were illustrated, focusing mainly on the differences with respect to the position of the sensors. We confirmed that the magnitude, instead of three axis vector information, may be more useful in the body sway analysis.

Published
2014

150. Combined effect of pulsed electromagnetic field and sound wave on In vitro and In vivo neural differentiation of human mesenchymal stem cells.

Author

Choi, Yun‐Kyong, Urnukhsaikhan, Enerelt, Yoon, Hee‐Hoon, Seo, Young‐Kwon, Cho, Hyunjin, Jeong, Jong‐Seob, Kim, Soo‐Chan, and Park, Jung‐Keug

Subjects
ELECTROMAGNETIC fields, PULSE circuits, SOUND waves, BONE marrow, MESENCHYMAL stem cells
Abstract

Biophysical wave stimulus has been used as an effective tool to promote cellular maturation and differentiation in the construction of engineered tissue. Pulsed electromagnetic fields (PEMFs) and sound waves have been selected as effective stimuli that can promote neural differentiation. The aim of this study was to investigate the synergistic effect of PEMFs and sound waves on the neural differentiation potential in vitro and in vivo using human bone marrow mesenchymal stem cells (hBM-MSCs). In vitro, neural-related genes in hBM-MSCs were accelerated by the combined exposure to both waves more than by individual exposure to PEMFs or sound waves. The combined wave also up-regulated the expression of neural and synaptic-related proteins in a three-dimensional (3-D) culture system through the phosphorylation of extracellular signal-related kinase. In a mouse model of photochemically induced ischemia, exposure to the combined wave reduced the infarction volume and improved post-injury behavioral activity. These results indicate that a combined stimulus of biophysical waves, PEMFs and sound can enhance and possibly affect the differentiation of MSCs into neural cells. Our study is meaningful for highlighting the potential of combined wave for neurogenic effects and providing new therapeutic approaches for neural cell therapy. © 2016 American Institute of Chemical Engineers Biotechnol. Prog., 33:201-211, 2017 [ABSTRACT FROM AUTHOR]

Published
2017
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