Analysis of the cornified cell envelope in lamellar ichthyosis.

Analysis of the cornified cell envelope in lamellar ichthyosis.
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层状鱼鳞病角质化细胞包膜的分析。

DOI:
10.1001/archderm.1993.01680260088013
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发表时间:
1993
影响因子:
--
通讯作者:
E. Frenk
E. Frenk
中科院分区:
--
文献类型:
--
作者:
D. Hohl;M. Huber;E. Frenk

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背景 洛丽蛋白和总蛋白是角化细胞膜的主要前体蛋白,在表皮分化后期表达。Involucrin在正常人表皮中的表达始于棘层的上层,先于Clouicrin的表达,后者仅限于颗粒层。随后,转谷氨酰胺酶TGK/E的活性使这两种蛋白质通过N-(γ-谷氨酰基)赖氨酸异肽键作为角化细胞被膜的主要成分而交联。在这项研究中,对3例板层状鱼鳞病进行了免疫组织学研究,抗体为氯化三氯乙酸甘油酯、毛壳素、微丝蛋白和转谷氨酰胺酶TGK。 观察 通过抗体B.C.1和K.D.3评估,氯化蛋白和总蛋白的高表达与转谷氨酰胺酶TGK的胞浆染色减弱是一致的。这种模式在出生时没有出现在火棉状婴儿身上,但在临床出现板层状鱼鳞病的表型前2周就出现了。 结论 结果表明:(1)谷氨酰胺转氨酶TGK的膜锚定干扰可改变角化细胞膜的角化和包膜的形成;(2)免疫组织学检查可作为火棉婴儿治疗的早期诊断和预后判断的工具。
BACKGROUND Loricrin and involucrin are major precursor proteins to the cornified cell envelope expressed late in epidermal differentiation. Involucrin expression starts in the upper spinous layers in normal human epidermis and precedes loricrin expression, which is restricted to the granular layer. Subsequently, both proteins become cross-linked by the activity of transglutaminases TGK/E as major components of the cornified cell envelope by N epsilon-(gamma-glutamyl)lysine isopeptide bonds. In this study, three cases of lamellar ichthyosis were analyzed by immunohistologic study with antibodies to loricrin, involucrin, filaggrin, and transglutaminase TGK. OBSERVATIONS A high expression of loricrin and involucrin with a peculiar and abnormal cytoplasmic staining concurred with a diminished cytoplasmic staining of transglutaminase TGK as assessed by antibodies B.C.1 and K.D.3. This pattern was absent in a collodion baby at birth but present 2 weeks later before a phenotype of lamellar ichthyosis appeared clinically. CONCLUSIONS The results suggest that in our cases of lamellar ichthyosis, (1) disturbed membrane anchorage of transglutaminase TGK could alter loricrin and involucrin cross-linkage and the formation of the cornified cell envelope and that (2) immunohistologic study might serve as an early diagnostic and prognostic tool in the treatment of collodion babies.
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