Редактирование: EFEMP1

EGF-containing fibulin-like extracellular matrix protein 1 precursor (Extracellular protein S1-5) (Fibrillin-like protein) (Fibulin-3) (FIBL-3) [FBLN3] [FBNL]

==Publications==

{{medline-entry
|title=A novel age-related venous amyloidosis derived from [[EGF]]-containing fibulin-like extracellular matrix protein 1.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/30565683
|abstract=Most intractable tissue-degenerative disorders share a common pathogenic condition, so-called proteinopathy. Amyloid-related disorders are the most common proteinopathies and are characterized by amyloid fibril deposits in the brain or other organs. Aging is generally associated with the development of these amyloid-related disorders, but we still do not fully understand how functional proteins become pathogenic amyloid deposits during the human aging process. We identified a novel amyloidogenic protein, named epidermal growth factor-containing fibulin-like extracellular matrix protein 1 ([[EFEMP1]]), in massive venous amyloid deposits in specimens that we obtained from an autopsied patient who died of gastrointestinal bleeding. Our postmortem analyses of additional patients indicate that [[EFEMP1]] amyloid deposits frequently developed in systemic venous walls of elderly people. [[EFEMP1]] was highly expressed in veins, and aging enhanced venous [[EFEMP1]] expression. In addition, biochemical analyses indicated that these venous amyloid deposits consisted of C-terminal regions of [[EFEMP1]]. In vitro studies showed that C-terminal regions formed amyloid fibrils, which inhibited venous tube formation and cell viability. [[EFEMP1]] thus caused a novel age-related venous amyloid-related disorder frequently found in the elderly population. Understanding [[EFEMP1]] amyloid formation provides new insights into amyloid-related disorders occurring during the aging process. Copyright © 2018 Pathological Society of Great Britain and Ireland. Published by John Wiley