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Biomedicines
Special Issues
Extracellular Matrix in Health and Disorders
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Extracellular Matrix in Health and Disorders

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: 31 May 2025 | Viewed by 4624

Special Issue Editor

Dr. Caterina Licini

E-Mail Website
Guest Editor
Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy
Interests: bone; osteoporosis; histology; biomedicine; extracellular matrix

Special Issue Information

Dear Colleagues,

The extracellular matrix (ECM) is a complex structure composed of proteins, glycoproteins, and proteoglycans in different percentages. This variability provides different functional and structural features to many tissues (dermis, bone, tendon, etc.), making the ECM a key point for tissue specialization and physiological processes.

Changes in protein composition and structure, as well as the structural modification of the ECM itself, lead to tissue dysfunctionality, such as photoaging and inadequate matrix repair, and diseases, such as osteoporosis.

Research aimed at clarifying the ECM’s role and qualities is essential for identifying specific targets, designing efficient drugs, and setting clinical strategies able to counter the features of pathological tissues.

This Special Issue aims to collect novel insights into the ECM’s involvement in the normal and altered physiological processes of the skin/dermis, bone, tendon, and other connective tissues.

We are interested in submissions of original research and reviews focusing on the in vivo and ex vivo characterization of the ECM in healthy and pathological tissues, with particular attention paid to structural analyses and advances in imaging, as well as in vitro studies and models, able to increase our knowledge concerning the ECM.

Dr. Caterina Licini
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • ECM
  • connective tissue
  • healthy tissues
  • pathological tissues
  • imaging
  • drug targets
  • markers
  • dermis
  • bone
  • tendon

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Published Papers (2 papers)

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Research

20 pages, 8903 KiB  
Article
Integrative Multi-Omics Approach in Vascular Ehlers–Danlos Syndrome: Further Insights into the Disease Mechanisms by Proteomic Analysis of Patient Dermal Fibroblasts
by Nicola Chiarelli, Valeria Cinquina, Nicoletta Zoppi, Valeria Bertini, Marianna Maddaluno, Chiara De Leonibus, Carmine Settembre, Marina Venturini, Marina Colombi and Marco Ritelli
Biomedicines 2024, 12(12), 2749; https://doi.org/10.3390/biomedicines12122749 - 30 Nov 2024
Cited by 1 | Viewed by 1910
Abstract
Background: Dominant mutations in COL3A1 are known to cause vascular Ehlers–Danlos syndrome (vEDS) by impairing extracellular matrix (ECM) homeostasis. This disruption leads to the fragility of soft connective tissues and a significantly increased risk of life-threatening arterial and organ ruptures. Currently, treatments [...] Read more.
Background: Dominant mutations in COL3A1 are known to cause vascular Ehlers–Danlos syndrome (vEDS) by impairing extracellular matrix (ECM) homeostasis. This disruption leads to the fragility of soft connective tissues and a significantly increased risk of life-threatening arterial and organ ruptures. Currently, treatments for vEDS are primarily symptomatic, largely due to a limited understanding of its underlying pathobiology and molecular mechanisms. Methods: In this study, we conducted a comprehensive analysis of the intracellular proteome of vEDS fibroblasts, integrating these findings with our previous transcriptome results to identify key molecular pathways that drive the disease. Additionally, we explored the therapeutic potential of inhibiting miR-29b-3p as a proof of concept. Results: Our integrative multi-omics analysis revealed complex pathological networks, emphasizing the critical role of miRNAs, particularly miR-29b-3p, in impairing ECM organization, autophagy, and cellular stress responses, all of which contribute to the pathogenesis of vEDS. Notably, the inhibition of miR-29b-3p in vEDS fibroblasts resulted in the upregulation of several differentially expressed target genes involved in these critical processes, as well as increased protein expression of essential ECM components, such as collagen types V and I. These changes suggest potential therapeutic benefits aimed at improving ECM integrity and restoring intracellular homeostasis. Conclusions: Overall, our findings advance our understanding of the complex biological mechanisms driving vEDS and lay a solid foundation for future research focused on developing targeted and effective treatment strategies for this life-threatening disorder. Full article
(This article belongs to the Special Issue Extracellular Matrix in Health and Disorders)
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25 pages, 3554 KiB  
Article
Xylosyltransferase-Deficiency in Human Dermal Fibroblasts Induces Compensatory Myofibroblast Differentiation and Long-Term ECM Reduction
by Anika Kleine, Matthias Kühle, Thanh-Diep Ly, Vanessa Schmidt, Isabel Faust-Hinse, Cornelius Knabbe and Bastian Fischer
Biomedicines 2024, 12(3), 572; https://doi.org/10.3390/biomedicines12030572 - 4 Mar 2024
Cited by 1 | Viewed by 1824
Abstract
Desbuquois dysplasia type 2 (DBQD2) and spondylo-ocular syndrome (SOS) are autosomal recessive disorders affecting the extracellular matrix (ECM) and categorized as glycosaminoglycan (GAG) linkeropathies. Linkeropathies result from mutations within glycosyltransferases involved in the synthesis of the tetrasaccharide linker, a linker between the core [...] Read more.
Desbuquois dysplasia type 2 (DBQD2) and spondylo-ocular syndrome (SOS) are autosomal recessive disorders affecting the extracellular matrix (ECM) and categorized as glycosaminoglycan (GAG) linkeropathies. Linkeropathies result from mutations within glycosyltransferases involved in the synthesis of the tetrasaccharide linker, a linker between the core protein of proteoglycan (PG) and GAG. DBQD2 and SOS are caused by the isolated mutations of the xylosyltransferase (XT) isoforms. In this work, we successfully generated XYLT1- as well as XYLT2-deficient GAG linkeropathy model systems in human dermal fibroblasts using a ribonucleoprotein-based CRISPR/Cas9-system. Furthermore, it was possible to generate a complete XYLT-knockdown. Short- and long-term XT activity deficiency led to the mutual reduction in all linker transferase-encoding genes, suggesting a potential multienzyme complex with mutual regulation. Fibroblasts compensated for ECM misregulation initially by overexpressing ECM through the TGFβ1 signaling pathway, akin to myofibroblast differentiation patterns. The long-term reduction in one XT isoform induced a stress response, reducing ECM components. The isolated XYLT1-knockout exhibited α-smooth muscle actin overexpression, possibly partially compensated by unaltered XT-II activity. XYLT2-knockout leads to the reduction in both XT isoforms and a strong stress response with indications of oxidative stress, induced senescence and apoptotic cells. In conclusion, introducing XYLT-deficiency revealed temporal and isoform-specific regulatory differences. Full article
(This article belongs to the Special Issue Extracellular Matrix in Health and Disorders)
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