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硬皮症 皮膚纖維芽細胞에서 Ⅶ형 膠原質 遺傳子의 發現 增加
申汶錫,宋俊榮 계명대학교 의과대학 1999 仁耕 宋俊榮 敎授 定年紀念 論文集 Vol.S No.-
Type Ⅶ collagen, homotrimer, [ 1 (Ⅶ)]3 and each chain consists of a central collagenous domain of 145-kD that is flanked by noncollageous domains. is a relatively low abundance extracellular matrix protein among the collagenous molecules. Among the minor collagens, type VII collagen has been demonstrated by immunolocalization studies to be component of anchoring fibrils and structures extending perpendicularly from the lamina densa to the upper papillary dermis. In this study, RNA was isolated from cultured scleroderma skin fibroblasts and the type Ⅶ collagen gene expression was examed using reverse transcriptase-PCR. The ratio of type VII collagen/GAPDH RT-PCR product were 2l. 7 -r. 7.6 in normal fibroblasts and 63.3±15.3 in scleroderma fibroblasts. To evaluate the expression of type VII collagen at the protein level, indirected immunofluorescence stain with anti-type Ⅶ collagen antibody was performed on scleroderma skin and cultured fibroblasts. Immunofluorescence staining with anti-type Ⅶ collagen monoclonal antibody in cultured normal and scleroderma fibroblast was showed the presence of a relatively faint staining in normal fibroblast, while the staining is markedly enhanced in scleroderma fibroblast. Immunofluorescence staining of normal skin or skin from patient with seleroderma with anti-type VII collagen monoclonal antibody was showed the strong linear staining for type VII collagen epitopes in the basememt membrane zone in all two specimens. These data suggest that type Ⅶcollagen which may be the main source of the sclerotic change of skin in scleroderma is contributed to the pathogenesis of the scleroderma.
硬皮症 皮膚纖維芽細胞에서 Ⅶ형 膠原質 遺傳子의 發現 增加
申汶錫,宋俊榮 계명대학교 醫科大學 皮膚科學 1999 논문집 Vol.S No.-
Type Ⅶ collagen, homotrimer, [ 1 (Ⅶ)]3 and each chain consists of a central collagenous domain of 145-kD that is flanked by noncollageous domains. is a relatively low abundance extracellular matrix protein among the collagenous molecules. Among the minor collagens, type VII collagen has been demonstrated by immunolocalization studies to be component of anchoring fibrils and structures extending perpendicularly from the lamina densa to the upper papillary dermis. In this study, RNA was isolated from cultured scleroderma skin fibroblasts and the type Ⅶ collagen gene expression was examed using reverse transcriptase-PCR. The ratio of type VII collagen/GAPDH RT-PCR product were 2l. 7 -r. 7.6 in normal fibroblasts and 63.3±15.3 in scleroderma fibroblasts. To evaluate the expression of type VII collagen at the protein level, indirected immunofluorescence stain with anti-type Ⅶ collagen antibody was performed on scleroderma skin and cultured fibroblasts. Immunofluorescence staining with anti-type Ⅶ collagen monoclonal antibody in cultured normal and scleroderma fibroblast was showed the presence of a relatively faint staining in normal fibroblast, while the staining is markedly enhanced in scleroderma fibroblast. Immunofluorescence staining of normal skin or skin from patient with seleroderma with anti-type VII collagen monoclonal antibody was showed the strong linear staining for type VII collagen epitopes in the basememt membrane zone in all two specimens. These data suggest that type Ⅶcollagen which may be the main source of the sclerotic change of skin in scleroderma is contributed to the pathogenesis of the scleroderma.
신문석,구본식,권호준,김병천,이규석 ( Moon Seok Sihn,Bon Sik Koo,Ho June Kwon,Byung Chun Kim,Kyu Suk Lee ) 대한피부과학회 1997 대한피부과학회지 Vol.35 No.3
Ecthyma Gangrenosum is a rapidly progressing skin infection charact,erized by edema, hemorragic bullae and necrosis. Ecthyma Gangrenosum occurs almost exclusively in severely imrnunocompromised patients during the course of pseudomanas aeruginosa septisemia. A 5 month-old boy was transferred to our department because of multiple well defined central necrotic black-colored large erythematous bullae. In the bacterial cultures of skin, stool and liver aspiration fluid, Pseudomonas aeruginosa was grown. In the abdominal ultrasonogram and computed tomogram, the multiple liver abscess, hepatomegaly and ascites were shown on both hepatic lobes. Herein we report a case of ecthyma gangrenosum associated with liver abscess. (Kor J Dermatol 1997;35(3): '.41-545)