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A Case of Infantile Alexander Disease Accompanied by Infantile Spasms Diagnosed by DNA Analysis
이정무,김애숙,이선주,조성민,이동석,최성민,김두권,기창석,김종원 대한의학회 2006 Journal of Korean medical science Vol.21 No.5
Alexander disease (AD) is a rare leukodystrophy of the central nervous system of unknown etiology. AD is characterized by progressive failure of central myelination and the accumulation of Rosenthal fibers in astrocytes, and is inevitably lethal in nature. Symptomatically, AD is associated with leukoencephalopathy with macrocephaly, seizures, and psychomotor retardation in infants, and usually leads to death within the first decade. Its characteristic magnetic resonance imaging (MRI) findings have been described as demyelination predominantly in the frontal lobe. Moreover, dominant mutations in the GFAP gene, coding for glial fibrillary acidic protein (GFAP), a principal astrocytic intermediate filament protein, have been shown to lead to AD. The disease can now be detected by genetic diagnosis. We report the Korean case of an 8-month-old male patient with AD. He was clinically characterized due to the presence of psychomotor retardation, megalencephaly, spasticity, and recurrent seizures including infantile spasms which is a remarkable presentation. Demyelination in the frontal lobe and in a portion of the temporal lobe was demonstrated by brain MRI. Moreover, DNA analysis of peripheral blood showed the presence of a R239L mutation in the GFAP gene, involving the replacement of guanine with thymine.
이정무,김건주,정윤교 한국공작기계학회 2002 한국공작기계학회 추계학술대회논문집 Vol.2002 No.-
Recently, in machining industry much progress has been made by taking advantage of high speed machining. On the other hand as disadvantage high speed machining involves shortening the life of cutting tool. In this research we want to evaluate the performance of appropriate endmill for high speed machining in accordance with surface roughness of land width and clearance angle of flat-endmill
Generation of Astrocyte-Specific MAOB Conditional Knockout Mouse with Minimal Tonic GABA Inhibition
이정무,사문선,안희영,Kim Jong Min Joseph,권재,윤보은,이창준 한국뇌신경과학회 2022 Experimental Neurobiology Vol.31 No.3
Monoamine oxidase B (MAOB) is a key enzyme for GABA production in astrocytes in several brain regions. To date, the role of astrocytic MAOB has been studied in MAOB null knockout (KO) mice, although MAOB is expressed throughout the body. Therefore, there has been a need for genetically engineered mice in which only astrocytic MAOB is targeted. Here, we generated an astrocyte-specific MAOB conditional KO (cKO) mouse line and characterized it in the cerebellar and striatal regions of the brain. Using the CRISPR-Cas9 gene-editing technique, we generated Maob floxed mice (B6-Maobem1Cjl /Ibs) which have floxed exons 2 and 3 of Maob with two loxP sites. By crossing these mice with hGFAP-CreERT2, we obtained Maob floxed::hGFAP-CreERT2 mice which have a property of tamoxifen-inducible ablation of Maob under the human GFAP (hGFAP) promoter. When we treated Maob floxed::hGFAP-CreERT2 mice with tamoxifen for 5 consecutive days, MAOB and GABA immunoreactivity were significantly reduced in striatal astrocytes as well as in Bergmann glia and lamellar astrocytes in the cerebellum, compared to sunflower oil-injected control mice. Moreover, astrocyte-specific MAOB cKO led to a 74.6% reduction in tonic GABA currents from granule cells and a 76.8% reduction from medium spiny neurons. Our results validate that astrocytic MAOB is a critical enzyme for the synthesis of GABA in astrocytes. We propose that this new mouse line could be widely used in studies of various brain diseases to elucidate the pathological role of astrocytic MAOB in the future.
강산성 용액의 pH를 측정할 수 있는 미셀기반의 형광센서 개발
이정무,이성호,Lee, Jeongmoo,Lee, Seoung Ho 한국환경과학회 2020 한국환경과학회지 Vol.29 No.8
In this study, an effective fluorescence pH sensor based on conjugated polyelectrolyte micelles (CPMs) was devised for detecting extremely acidic conditions. An amphiphilic coumarin derivative (CC12-N), a building block, was prepared, into which an ionizable amino group, aryl amine, was incorporated as a potential hydrophilic moiety. This monomer displays self-assembled micelle formation in extremely acidic pH ranges, giving a hydrophobic π-extended conjugated system at the inner part and hydrophilic functionality at the periphery, resulting in efficient fluorescence intensity enhancement. This new micelle-based fluorescence provides an efficient sensing platform for detecting very low pH values in the presence of competing substances.