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皮肤松弛症成纤维细胞中VI型胶原蛋白基因表达增加。

Increased expression of type VI collagen genes in cutis laxa fibroblasts.

作者信息

Hatamochi A, Arakawa M, Mori K, Mori Y, Ueki H, Yoshioka H

机构信息

Department of Dermatology, Kawasaki Medical School, Kurashiki, Japan.

出版信息

J Dermatol Sci. 1996 Feb;11(2):97-103. doi: 10.1016/0923-1811(95)00425-4.

DOI:10.1016/0923-1811(95)00425-4
PMID:8869029
Abstract

Type VI collagen gene expression in cutis laxa was studied by measuring messenger RNA (mRNA) and protein production levels in four fibroblast strains from patients with congenital cutis laxa and comparing them with those in fibroblasts obtained from age-matched healthy subjects. Levels of type VI collagen mRNA were increased in all cutis laxa fibroblast strains and the levels of alpha 1 (VI) and alpha 3 (VI) chain mRNAs increased in parallel. Increases in type VI collagen mRNAs correlated well with production levels of the corresponding proteins, as determined by immunological assay. These results suggest that increased type VI collagen gene expression is one of the characteristics of cutis laxa dermal fibroblasts and that this abnormality may be related to the skin changes in cutis laxa.

摘要

通过测量先天性皮肤松弛症患者的四个成纤维细胞株中信使核糖核酸(mRNA)和蛋白质产生水平,并将其与从年龄匹配的健康受试者获得的成纤维细胞中的水平进行比较,研究了皮肤松弛症中VI型胶原基因的表达。所有皮肤松弛症成纤维细胞株中VI型胶原mRNA水平均升高,α1(VI)和α3(VI)链mRNA水平平行升高。如通过免疫测定所确定的,VI型胶原mRNA的增加与相应蛋白质的产生水平密切相关。这些结果表明,VI型胶原基因表达增加是皮肤松弛症真皮成纤维细胞的特征之一,并且这种异常可能与皮肤松弛症中的皮肤变化有关。

相似文献

1
Increased expression of type VI collagen genes in cutis laxa fibroblasts.皮肤松弛症成纤维细胞中VI型胶原蛋白基因表达增加。
J Dermatol Sci. 1996 Feb;11(2):97-103. doi: 10.1016/0923-1811(95)00425-4.
2
Collagen metabolism in cutis laxa fibroblasts: increased collagenase gene expression associated with unaltered expression of type I and type III collagen.皮肤松弛症成纤维细胞中的胶原蛋白代谢:胶原酶基因表达增加,而I型和III型胶原蛋白表达未改变。
J Invest Dermatol. 1991 Sep;97(3):483-7. doi: 10.1111/1523-1747.ep12481519.
3
Autosomal recessive cutis laxa syndrome. A case report.常染色体隐性遗传性皮肤松弛综合征。病例报告。
Acta Derm Venereol. 1996 Jul;76(4):298-301. doi: 10.2340/0001555576298301.
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Heterogeneity of elastin expression in cutis laxa fibroblast strains.皮肤松弛症成纤维细胞株中弹性蛋白表达的异质性。
J Invest Dermatol. 1989 Jul;93(1):147-53. doi: 10.1111/1523-1747.ep12277389.
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Cutis laxa: reduced elastin gene expression in skin fibroblast cultures as determined by hybridizations with a homologous cDNA and an exon 1-specific oligonucleotide.皮肤松弛症:通过与同源cDNA和外显子1特异性寡核苷酸杂交测定,皮肤成纤维细胞培养物中弹性蛋白基因表达降低。
J Biol Chem. 1988 May 15;263(14):6465-7.
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Elastin production and degradation in cutis laxa acquisita.获得性皮肤松弛症中的弹性蛋白生成与降解
J Invest Dermatol. 1994 Oct;103(4):583-8. doi: 10.1111/1523-1747.ep12396893.
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A disease with features of cutis laxa and Ehlers-Danlos syndrome. Report of a mother and daughter.一种具有皮肤松弛症和埃勒斯-当洛综合征特征的疾病。一位母亲和女儿的病例报告。
Hum Genet. 1988 Jan;78(1):9-12. doi: 10.1007/BF00291225.
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Decreased type VI collagen gene expression in cultured Werner's syndrome fibroblasts.培养的沃纳综合征成纤维细胞中VI型胶原蛋白基因表达降低。
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Highly variable cutis laxa resulting from a dominant splicing mutation of the elastin gene.由弹性蛋白基因的显性剪接突变导致的高度可变型皮肤松弛症。
Am J Med Genet A. 2008 Apr 15;146A(8):977-83. doi: 10.1002/ajmg.a.32242.
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Severe congenital cutis laxa with cardiovascular manifestations due to homozygous deletions in ALDH18A1.严重先天性皮肤松弛症伴心血管表现,归因于 ALDH18A1 纯合缺失。
Mol Genet Metab. 2014 Aug;112(4):310-6. doi: 10.1016/j.ymgme.2014.05.003. Epub 2014 May 21.

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