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Fibulins: physiological and disease perspectives.


ABSTRACT: The fibulins are a family of proteins that are associated with basement membranes and elastic extracellular matrix fibres. This review summarizes findings from studies of animal models of fibulin deficiency, human fibulin gene mutations, human tumours and injury models that have advanced our understanding of the normal and pathological roles of members of this formerly obscure family.

SUBMITTER: Argraves WS 

PROVIDER: S-EPMC1326425 | biostudies-literature | 2003 Dec

REPOSITORIES: biostudies-literature

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Fibulins: physiological and disease perspectives.

Argraves W Scott WS   Greene Lisa M LM   Cooley Marion A MA   Gallagher William M WM  

EMBO reports 20031201 12


The fibulins are a family of proteins that are associated with basement membranes and elastic extracellular matrix fibres. This review summarizes findings from studies of animal models of fibulin deficiency, human fibulin gene mutations, human tumours and injury models that have advanced our understanding of the normal and pathological roles of members of this formerly obscure family. ...[more]

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