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肥大细胞增多症:皮肤病理生理学的新认识

Mastocytosis: new understandings in cutaneous pathophysiology.

作者信息

Lazarus G S

机构信息

Office of the Dean, School of Medicine, University of California, Davis 95616, USA.

出版信息

J Dermatol. 1996 Nov;23(11):769-72. doi: 10.1111/j.1346-8138.1996.tb02699.x.

DOI:10.1111/j.1346-8138.1996.tb02699.x
PMID:8990699
Abstract

Human mast cells contain large quantities of chymotryptic and tryptic proteinases. In human skin, mast cells contain both chymase and tryptase, whereas, in the mucosa of the gastrointestinal tract, mast cells contain primarily tryptase. By contrast, submucosal mast cells in the gastrointestinal tract are of the connective tissue type because they contain both chymase and tryptase. Chymase has a broad array of biological functions which include; activation of angiotensin, cleavage of basement membrane through the lamina lucida, activation of IL1 beta, and potentiation of histamine. Chymase may play a significant role in the control of a variety of biological phenomena. Urticaria pigmentosa is a disease characterized by deposition of "normal" connective tissue mast cells within the skin. The source of these mast cells is the bone marrow and mast cells appear to be deposited within other internal organs in almost all cases. Excretion of histamine and prostaglandin metabolites correlates with the deposition of mast cells in extracutaneous sites. High potency steroids under occlusion for six week results in long-lasting clearing of the cutaneous lesions with minimal side effects.

摘要

人类肥大细胞含有大量的糜蛋白酶和胰蛋白酶。在人类皮肤中,肥大细胞同时含有糜酶和组织蛋白酶,而在胃肠道黏膜中,肥大细胞主要含有组织蛋白酶。相比之下,胃肠道黏膜下肥大细胞属于结缔组织型,因为它们同时含有糜酶和组织蛋白酶。糜酶具有广泛的生物学功能,包括激活血管紧张素、通过透明板裂解基底膜、激活白细胞介素1β以及增强组胺作用。糜酶可能在多种生物学现象的调控中发挥重要作用。色素性荨麻疹是一种以“正常”结缔组织肥大细胞在皮肤内沉积为特征的疾病。这些肥大细胞来源于骨髓,几乎在所有病例中,肥大细胞似乎都沉积在其他内脏器官中。组胺和前列腺素代谢产物的排泄与肥大细胞在皮肤外部位的沉积相关。强效类固醇封闭治疗六周可使皮肤病变长期消退,且副作用最小。

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Mastocytosis: new understandings in cutaneous pathophysiology.肥大细胞增多症:皮肤病理生理学的新认识
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Determination of the primary structures of human skin chymase and cathepsin G from cutaneous mast cells of urticaria pigmentosa lesions.从色素性荨麻疹皮损的皮肤肥大细胞中测定人皮肤糜蛋白酶和组织蛋白酶G的一级结构。
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Mast-cell phenotype in indolent forms of mastocytosis. Ultrastructural features, fluorescence detection of avidin binding, and immunofluorescent determination of chymase, tryptase, and carboxypeptidase.惰性肥大细胞增多症中的肥大细胞表型。超微结构特征、抗生物素蛋白结合的荧光检测以及糜蛋白酶、组织蛋白酶和羧肽酶的免疫荧光测定。
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Altered expression of mast cell chymase and tryptase and of c-Kit in human cutaneous scar tissue.人皮肤瘢痕组织中肥大细胞糜蛋白酶、组织蛋白酶和c-Kit的表达改变。
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No relationship between histamine release measured as metabolite excretion in the urine, and serum levels of mast cell specific tryptase in mastocytosis.在肥大细胞增多症中,以尿中代谢物排泄量衡量的组胺释放与肥大细胞特异性类胰蛋白酶的血清水平之间无相关性。
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Mast cells expressing chymase but not tryptase can be derived by culturing human progenitors in conditioned medium obtained from a human mastocytosis cell strain with c-kit ligand.通过在从具有c-kit配体的人肥大细胞增多症细胞系获得的条件培养基中培养人祖细胞,可以获得表达糜蛋白酶但不表达组织蛋白酶的肥大细胞。
J Immunol. 1996 Jun 15;156(12):4839-44.

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