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Last update 08 May 2025

EFEMP2

Last update 08 May 2025

Basic Info

Synonyms

EFEMP2, EGF containing fibulin extracellular matrix protein 2, EGF-containing fibulin-like extracellular matrix protein 2

+ [5]

Introduction

Plays a crucial role in elastic fiber formation in tissue, and in the formation of ultrastructural connections between elastic laminae and smooth muscle cells in the aorta, therefore participates in terminal differentiation and maturation of smooth muscle cell (SMC) and in the mechanical properties and wall integrity maintenance of the aorta (PubMed:27339457). In addition, is involved in the control of collagen fibril assembly in tissue throught proteolytic activation of LOX leading to cross- linking of collagen and elastin (By similarity). Also promotes ELN coacervation and participates in the deposition of ELN coacervates on to microfibrils but also regulates ELN cross- linking through LOX interaction (PubMed:18973305, PubMed:19570982). Moreover adheres to the cells through heparin binding in a calcium-dependent manner and regulates vascularlar smooth muscle cells proliferation through angiotensin signaling (PubMed:23782690).

Drugs associated with EFEMP2

HG-1241

Target

EFEMP2

Mechanism

EFEMP2 gene modulators [+1]

Active Org.-

Originator Org.

Human Genome Sciences, Inc.

Active Indication-

Inactive Indication

Neoplasms

Drug Highest PhasePending

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with EFEMP2

100 Translational Medicine associated with EFEMP2

0 Patents (Medical) associated with EFEMP2

199

Literatures (Medical) associated with EFEMP2

15 Apr 2025·The FASEB Journal

Fibulin‐4 and latent‐transforming growth factor beta‐binding protein‐4 interactions with syndecan‐2 and syndecan‐3 are required for elastogenesis

Article

Author: Nelea, Valentin ; Ricard‐Blum, Sylvie ; Reinhardt, Dieter P. ; Lamarche‐Vane, Nathalie ; Dinesh, Neha E. H. ; Hakami, Hana

AbstractElastogenesis is a cell surface‐located hierarchical process that requires the core components tropoelastin and fibrillins and several accessory proteins, including fibulin‐4 (FBLN4) and latent TGF‐β binding protein‐4 (LTBP4). FBLN4 and LTBP4 interact with cells, but their cell receptors and associated molecular elastogenic mechanisms remain unknown. Primary skin fibroblasts and several vascular smooth muscle cells bound strongly to FBLN4 multimers and LTBP4 monomers. We identified two cell interaction epitopes on FBLN4 located in cbEGF2‐3 and the C‐terminal domain, whereas FBLN4 multimerization sites were mapped to cbEGF4‐5 and the C‐terminal domain. We also determined a novel cell interaction site in the N‐terminal half of LTBP4. Cell binding to FBLN4 and LTBP4 was strongly inhibited in the presence of heparin, heparan sulfate, or after enzymatic removal of heparan sulfate, suggesting heparan sulfate proteoglycans as relevant cell surface receptors. siRNA knockdown experiments identified syndecan (SDC)2 and SDC3 as cell receptors for FBNL4 and SDC3 for LTBP4. Direct protein interactions between FBLN4 and the recombinant ectodomains of SDC2 and SDC3, and between LTBP4 and SDC3 validated these results. Interaction of the elastogenic cells with FBLN4 and LTBP4 enhanced elastogenesis, whereas SDC2 and/or SDC3 knockdowns led to reduced elastic fiber formation. The cell interactions with FBLN4 and LTBP4 significantly enhanced focal adhesion formation, induced cell contraction, and led to activation of focal adhesion kinase (FAK), Erk1/2, and RhoA. Pharmacological inhibition of these effectors markedly attenuated elastic fiber formation, and siRNA knockdown of SDC2 and SDC3 led to reduced levels of pFAK, pERK, and active RhoA. Together, these data demonstrate that FBLN4 and LTBP4 cell interactions through SDC2 and SDC3 promote elastogenesis by enhancing focal adhesion formation, leading to cell contractility through FAK, Erk1/2, and RhoA activation, underscoring the significance of these pathways in elastogenesis.

01 Apr 2025·Pathology - Research and Practice

Overexpression of MFAP5 inhibits the progression of papillary thyroid cancer and aerobic glycolysis by regulating the EFEMP2/Wnt/β-catenin pathway

Article

Author: Sun, Yihan ; Han, Yifan ; Li, Jiante ; Zhang, Yi ; Dong, Jianda

This study aimed to investigate the mechanism and role of MFAP5 in papillary thyroid carcinoma (PTC), laying the foundation for future clinical treatment of PTC. Using WB and RT-qPCR to determine MFAP5 expression in PTC tissues and paracancerous tissues, as well as human normal thyroid cell lines and human PTC cell lines. Viral infection of PTC cells by overexpressing MFAP5 and knocking down EFEMP2. CCK8 and a colony formation assay were used to assess PTC cell proliferation. Kits detect glucose uptake, lactate production, and WB analyses of GLUT1, HK-II, and LDHA expression to evaluate aerobic glycolysis. Nude mice were used as xenograft models for tumor growth assessment. MFAP5. WB detects the expression of EFEMP2, Myc, cyclin D1 and β-catenin. MFAP5 expression is significantly reduced in PTC tissues and cells. MFAP5 overexpression inhibits PTC cell proliferation and aerobic glycolysis. MFAP5 overexpression activates EFEMP2 and suppresses the Wnt/β-catenin pathway. Knockdown of EFEMP2 reverses PTC cell proliferation and aerobic glycolysis. Tumor growth can be inhibited in vivo by MFAP5 overexpression, which regulates the EFEMP2/Wnt/β-catenin pathway. Overexpression of MFAP5 inhibits PTC progression and aerobic glycolysis by regulating the EFEMP2/Wnt/β-catenin pathway.

01 Feb 2025·Thrombosis and Haemostasis

Novel Insights into the Aortic Mechanical Properties of Mice Modeling Hereditary Aortic Diseases

Article

Author: Csonka, Máté ; Sugiyama, Kaori ; Matyas, Gabor ; Sheppard, Mary B. ; Ferenczi, Szilamér ; Smith, Jeffrey D. ; Meienberg, Janine ; Dubacher, Nicolo ; Lamberti, Lisa ; Yanagisawa, Hiromi ; Caspar, Sylvan M. ; Nussbaumer, Vanessa ; Kovács, Krisztina J.

Objective Hereditary aortic diseases (hADs) increase the risk of aortic dissections and ruptures. Recently, we have established an objective approach to measure the rupture force of the murine aorta, thereby explaining the outcomes of clinical studies and assessing the added value of approved drugs in vascular Ehlers–Danlos syndrome (vEDS). Here, we applied our approach to six additional mouse hAD models. Material and Methods We used two mouse models (Fbn1C1041G and Fbn1mgR ) of Marfan syndrome (MFS) as well as one smooth-muscle-cell-specific knockout (SMKO) of Efemp2 and three CRISPR/Cas9-engineered knock-in models (Ltbp1, Mfap4, and Timp1). One of the two MFS models was subjected to 4-week-long losartan treatment. Per mouse, three rings of the thoracic aorta were prepared, mounted on a tissue puller, and uniaxially stretched until rupture. Results The aortic rupture force of the SMKO and both MFS models was significantly lower compared with wild-type mice but in both MFS models higher than in mice modeling vEDS. In contrast, the Ltbp1, Mfap4, and Timp1 knock-in models presented no impaired aortic integrity. As expected, losartan treatment reduced aneurysm formation but surprisingly had no impact on the aortic rupture force of our MFS mice. Conclusion Our read-out system can characterize the aortic biomechanical integrity of mice modeling not only vEDS but also related hADs, allowing the aortic-rupture-force-focused comparison of mouse models. Furthermore, aneurysm progression alone may not be a sufficient read-out for aortic rupture, as antihypertensive drugs reducing aortic dilatation might not strengthen the weakened aortic wall. Our results may enable identification of improved medical therapies of hADs.

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EFEMP2

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