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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.
CRISPR/Cas 지놈 편집 기술을 이용한 빠른 세대전환 유전자 조작 생쥐 생성
이희우 ( Hee Woo Lee ),성정준 ( Jung Joon Sung ),김혜민 ( Hye Min Kim ),이재삼 ( Jae Sam Lee ) 서울대학교 인구의학연구소 2014 人口醫學硏究論集 Vol.27 No.-
지난 30년 동안, 유전자 변형 마우스는 생체 내에서 유전자의 기능 연구에 매우 중요한 도구가 되었다. 생쥐의 지놈 변이를 위해 배아줄기세포내에서 유전자표적화 방법은 인간 질병에 중요한 정보를 제공해왔으며 약물 발견 및 표적 검증을 위한 플랫폼 역할을 해왔다. 최극에 널리 알려진 유전자편집 방법중 clustered regularly interepaced short palindromic repears(CRISPRs) and CRISPR-associated (Cas)[CRISPR/Cas]기반의 유전자편집은 생쥐와 쥐 등에 정확한 유전적 변화를 유도하기 위한 새로운 기술로 각광 받고 있다. 인간질병 모델로서 유전자 조작 마우스의 중요성을 감안 할 때. CRISPR은 유전자 변이를 통해 줄기세포와 체세포의 질병 관련 유전자를 제거하거나 교정하는 새로운 치료법 개발에 매우 유용할 것이다. 따라서 본 종설에서는 새로운 동물모델 생성에 있어 매우 혁신적인 장을 마련 할 수 있고, 또한 의생명과학 연구를 촉직 할 수 있는 가능성을 충분히 가진 CRISPR/Cas 유전자편집에 대하여 자세히 살펴보고자 한다.
유대훈 ( Dae Hoon Yoo ),송슬애 ( Seul Ae Song ),성정준 ( Jung Joon Sung ),조명수 ( Myung Soo Cho ) 서울대학교 인구의학연구소 2014 人口醫學硏究論集 Vol.27 No.-
All muscle movements including breathing, walking, and fine motor skills rely on the function of the spinal motor neuron to transmit signals from the brain to individual muscle groups. Motor neuron diseases (MNDs) result from the progressive degeneration and deat of motor neurons. The two most studied MNDs are spinal muscular acrophy (SMA), a childhood genetic disease, and amyotrophic lateral sclerosis (ALS), a adult-onset neurodegenerative disease. Both diseases involve neuromuscular dysfunction progressively leading to fatal paralysis. The potential of human pluripotent stem cells to treat patients with neurodegenerative disease is enormous. Stem cells can be introduced into clinical applications in several different ways, such as disease modeling, drug screening, and cell replacement therapy. Even though it may take quite a long time to address some problematice, the establishment and optimization of human stem cell differentiation protocols for clinical applications may hold the key to cure diverse disorders.
윤병남 ( Byung Nam Yoon ),최성혜 ( Seong Hye Choi ),나정호 ( Joung Ho Rha ),성정준 ( Jung Joon Sung ),마은주 ( Eun Ju Ma ),이광우 ( Kwang Woo Lee ) 대한외상학회 2014 大韓外傷學會誌 Vol.27 No.3
The brachial plexus is a network of nerves that provides movement and feeling to the shoulder, armand hand. The majority of acute brachial plexus injuries occur when the plexus is stretched violently or torn. This happens as result of the shoulder being pressed down forcefully while the head is pushed up and away from that shoulder. Such injuries frequently result from automobile or motor-cycle accidents or from falls and usually affect one side. Nerve injuries vary in severity from a mild stretching of the nerve to a tearing of the nerve root away from the spinal cord. We experienced a 50-year-old woman with weakness in both upper extremities after an attempted hanging. A consecutive workup revealed bilateral brachial plexus injuries. Six months after the incident, she had fully recovered. This is a very rare case of bilateral brachial plexus injuries after an attempted hanging. [ J Trauma Inj 2014;27:79-83 ]
인간 전분화능줄기세포를 이용한 세포치료제의 임상시험 현황
조명수 ( Myung Soo Cho ),유대훈 ( Dae Hoon Yoo ),송슬애 ( Seul Ae Song ),성정준 ( Jung Joon Sung ) 서울대학교 인구의학연구소 2014 人口醫學硏究論集 Vol.27 No.-
Human pluripotent stem cells including human embryonic stem cells and induced pluripotent stem cell can potentially be used for cell replacement therapies after the differentiation into various cell types although some remaining risk of cell therapeutics, clinical trials for pluripotent stem cell-based therapies is proceeding carefully. At present, applicable cell types and disease are limited. However, expanded clinical application is expected in the next few years. In this review, we will introduce a current state of ongoing clinical trials and refer to current and future perspectives for cell replacement therapy using human pluripotent stem cells.