The role of calcium in the organization of fibrillin microfibrils

FEBS Letters

Kielty, Cay M.¹  Shuttleworth, C.Adrian¹ 

[1] School of Biological Sciences, Division of Biochemistry, University of Manchester, Manchester, M13 9PT, UK

关键词: Fibrillin;    Calcium;    EGF motif;    Microfibril;   

DOI  :  10.1016/0014-5793(93)80829-J

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

The microfibrillar glycoprotein fibrillin has a multidomain structure which contains forty-three epidermal growth factor-like motifs with calciumbinding consensus sequences. We have utilized intact microfibrils isolated from human dermal fibroblast cultures to investigate the putative influence of bound calcium on microfibrillar organization and integrity. Incubation with EDTA or EGTA rapidly resulted in gross disruption of microfibril morphology. The treatment induced disorganization of the interbead domains although the regular beaded arrangement was always apparent. These changes were readily reversible on replacing calcium, indicating that the treatment had not compromised microfibrillar integrity. The data localize calcium binding EGF-like repeats to the interbead domains and indicate that lateral packing of fibrillin monomers is calcium-dependent. This arrangement suggests how mutations in epidermal growth factor-like domains of fibrillin might cause the disruption in microfibril organization and interactions which underlies the clinical symptoms of some Marian syndrome patients.

【 授权许可】

Unknown