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인간 유도만능줄기세포의 ALS 치료 적용에 관한 최신 연구 동향
고명성 ( Myung Sung Ko ),김대환 ( Dae Hwan Kim ),구승엽 ( Seung Yup Ku ),성정준 ( Jung Jun Sung ) 서울대학교 인구의학연구소 2012 人口醫學硏究論集 Vol.25 No.-
Induced pluripotent stem cells (iPSCs) are useful materials for cell therapy for various diseases including neuronal diseases. Amyotrophic lateral sclerosis (ALS), caused by loss of motor neuron, is one of the neurodegenerative diseases and leads to paralysis and death. Now, many research groups try to use iPSCs, which ate generated from patient`s own somatic cells, for a rescue treatment in ALS. Here, various current studies show the possible cell treatment application using the various cell types, motor neuron, astrocyte and microglia differentiated from patient specific iPSCs. In the further study, the transplantation of mixed cell types differentiated from iPSCs could be used as more efficient rescue system for ALS disease.
실험적 자가면역성 말초신경염에서의 조직병리적 병변 및 CD<sub>5</sub><sup>+</sup> B-림프구의 발현
조중양,최원준,김성훈,성정준,김호진,박경석,최기영,김현정,이광우,Cho, Joong-Yang,Choi, Won-Jun,Kim, Sung-Hun,Sung, Jung-Joon,Kim, Ho-Jin,Park, Kyung-Seok,Choi, Ki-Young,Kim, Hyun-Jung,Lee, Kwang-Woo 대한임상신경생리학회 1999 Annals of Clinical Neurophysiology Vol.1 No.2
Background : The pathogenesis of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), Guillain Barre syndrome (GBS) is not clear, but it has been known that the immune mechanisms play an important role. Authors performed this study to establish an animal model of experimental allergic neuritis (EAN) by immunizing the myelin components of peripheral nerves and to understand the electrophysiological and histopathological features as well as the ${CD_5}^+$ B-lymphocyte changes in peripheral bloods in the EAN models. Methods : Lewis rats weighing 150-200 gm were injected subcutaneously in soles two times with total myelin, P0, P1, or P2 proteins purified from the bovine cauda eguina. The EAN induction was assessed by evaluating clinical manifestations. The electrophysiological and histopathological features were studied as routine methods. The ${CD_5}^+$ Blymphocytes were double stained using monoclonal FITC conjugated anti-rat CD45RA and R-PE conjugated anti-rat ${CD_5}^+$ antibodies and calculated using a fluorescence activated cell sorter (FACS). Results : The EAN animal models were established. In two out of five, in one out of two, in none out of three, and in none out of one Lewis rats injected with purified total myelin, P0, P1, P2 proteins respectively, They showed slow spontaneous motor activity and weak resistance against pulling back by tails. The typical electrophysiological and histologic findings in total protein and P0 induced EAN animal models were the decreased conduction velocity, the decreased compound muscle action potential (CMAP) amplitude and the dispersion phenomenon. The perivascular infiltrates of lymphocytes with focal demyelinating process were found in light microscopy. The ${CD_5}^+$ B-lymphocyte expression in three EANs were 2.38%, 3.50% 2.50%, which were not significantly increased, compared with those in normal controls. Conclusion : The EAN animal models were successfully established by injecting the total myelin and P0 myelin and they showed electrophysiological and histological features typical of demyelinating process. However they did not show an increased expression of ${CD_5}^+$ B-lymphocyte in peripheral bloods which could be indirect evidence of humoral autoimmunity.
김대경(Dea Kyung Kim),이준영(Jun Young Lee),성정준(Jung Jun Sung),김의태(Eui-Tae Kim),김용식(Yong Sik Kim),권오상(Oh Sang Kwon),윤영철(Young Chul Yun),이태진(Tae Jin Lee),강용구(Yong Koo Kang),정윤희(Yoon Hee Chung),김성수(Sung Su Ki 대한해부학회 2004 Anatomy & Cell Biology Vol.37 No.6
기억력과 학습력의 개선은 두뇌의 기능적 향상 뿐 아니라, 신경의 보호차원에서도 그 의학적 가치가 크다. 스트레스에 의한 뇌기능 저하등과 같은 다양한 원인으로 인하여 기억력 감퇴가 일어날 수 있다. 또한 최근 기억력 감퇴 손상 억제 기전의 연구는 현저한 기능감소를 보이고 있는 콜린성 신경계의 기능을 보충해 주는데 초점이 맞추어져 있다. 이에 근거하여 본 연구에서는 가잠(Bombyx mori)으로부터 추출한 BF-7이 실제로 생체 내에서 감퇴된 기억력을 개선시켜줄 수 있는지를 검색하기 위하여 무스카리닉(muscarinic) 수용체의 길항제인 스코폴라민(scopolamine)을 투여하여 만든 기억력감퇴 세포와 동물모델을 사용하여 실험을 수행하였다. BF-7을 전처치하고 스코폴라민을 투여하였을 때, 세포생존율, passive avoidance test와 water maze test를 실시한 결과, BF-7이 매우 효과적으로 기억력을 개선시켜주고 있음이 나타났다. 이와 같은 결과는 이물질이 실제로 BBB (blood brain barrier)를 통과하여 뇌에서 시냅스의 콜린성 신경계의 기능을 높여주었으며, 두뇌기능 개선효과에 중요한 역할을 한다는 것을 시사한다. 또한 인지기능장애 정도를 평가할 수 있는 MMSE-K 임상검사법을 시행한 결과, BF-7을 섭취한 후, 획기적인 인지기능의 개선과 향상효과가 나타났다. 이는 BF-7이 인지기능을 개선해 줄 수 있는 안전하고 효과적인 후보물질로 개발할 만한 충분한 가치를 제공하여 주는 것이라고 사료된다. Various factors such as senescence, stress, neurodegenerative diseases including Alzheimer’s disease (AD) contribute to the impairments of organs, especially brain. Also, they should be negative factors on normal brain function, like as memory and cognition. In this study, the neuroprotective role of BF-7, extracted from Bombyx mori, was examined agaist scopolamine-induced neurotoxicity in SK-N-SH cells. In order to know if the BF-7 has positive role on the cognition and memory, we examined using SD rat model and human. Scopolamine-induced memory impairments were observed, as measured by the passive avoidance and water maze tests, but treatment with BF-7 significantly improved memory and cognitive function. Moreover, the memory index and memory preservation of clinical experiments using MMSE-K tests were significantly improved memory and cognitive function. This results strongly represent that the BF-7 play effectively positive role in the improvement of brain function including learning and memory. Taken together, our results suggested that the BF-7 should be useful for developing strategies protecting nervous system and improving brain function.
증례 : 류마티스 ; 만성 염증성 탈수초성 다발신경병증과 헤노흐-쇤라인 자반증을 동반한 강직성 척추염 1예
최병용 ( Byoong Yong Choi ),권현미 ( Hyun Mi Kwon ),성정준 ( Jung Jun Sung ),박성혜 ( Seong He Park ),송영욱 ( Yeong Wook Song ) 대한내과학회 2012 대한내과학회지 Vol.83 No.4
Peripheral nervous system dysfunction is a rare complication in Henoch-Schonlein purpura, but it tends to recover spontaneously without treatment. A 78-year-old man who had ankylosing spondylitis presented with Henoch-Schonlein purpura associated with progressive sensorimotor polyneuropathy. He was diagnosed with chronic inflammatory demyelinating polyneuropathy, which did not improve despite intravenous immunoglobulin therapy. We describe a case of Henoch-Schonlein purpura, accompanied by chronic inflammatory demyelinating polyneuropathy in a patient with ankylosing spondylitis. (Korean J Med 2012;83:538-542)