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FBLN5 Q9UBX5

弹性蛋白样蛋白-5 (FIBL-5) (发育动脉和神经 crest EGF样蛋白) (Dance) (尿液p50蛋白) (UP50)

蛋白质信息 (UniProt)

弹性蛋白样蛋白-5 (FIBL-5) (发育动脉和神经 crest EGF样蛋白) (Dance) (尿液p50蛋白) (UP50)

Q9UBX5

功能描述

对弹性纤维形成至关重要,参与连续弹性蛋白(ELN)聚合物的组装,并促进微原纤维与ELN的相互作用 (PubMed:18185537)。在皮肤、肺和脉管系统中稳定并组织弹性纤维 (By similarity)。通过整合素与RGD基序的相互作用促进内皮细胞的粘附。整合素受体的血管配体,可能在血管发育和重塑中发挥作用 (PubMed:10428823)。可能作为适配体介导FBN1与ELN之间的相互作用 (PubMed:17255108)。 {ECO:0000250|UniProtKB:Q9WVH9, ECO:0000269|PubMed:10428823, ECO:0000269|PubMed:17255108, ECO:0000269|PubMed:18185537}。

组织特异性

Expressed in skin fibroblasts (at protein level) (PubMed:17035250). Expressed predominantly in heart, ovary, and colon but also in kidney, pancreas, testis, lung and placenta. Not detectable in brain, liver, thymus, prostate, or peripheral blood leukocytes (PubMed:10428823).

亚细胞定位

Secreted

关键词

Age-related macular degeneration Calcium Cell adhesion Charcot-Marie-Tooth disease Disease variant Disulfide bond EGF-like domain Extracellular matrix Glycoprotein Neurodegeneration