Human Extracellular Matrix in Homeostasis and Pathology

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Cell Biology and Pathology".

Deadline for manuscript submissions: closed (30 September 2023) | Viewed by 4475

Special Issue Editors


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Guest Editor
Centre for Genomics and Personalised Health, Genomics Research Centre, School of Biomedical Sciences, Max Planck Queensland Centre, Queensland University of Technology (QUT), Queensland, 60 Musk Ave., Kelvin Grove, Brisbane, QLD 4059, Australia
Interests: extracellular matrix; proteoglycans; lineage specification; adult neurogenesis; microenvironment; initiation and progression of disease

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Guest Editor
Ikerbasque Research Associate, Leader of the Bioengineering in Regeneration and Cancer Group, Biodonostia Health Research Institute, Paseo Doctor Beguiristain s/n, 200014 San Sebastián, Spain
Interests: biomaterials; extracellular matrix; tissue engineering; regenerative medicine; cancer and bone metastasis

Special Issue Information

Dear Colleagues,

It is well established that complex intercellular interactions, as well as biomolecular and biomechanical cues from the extracellular matrix, dictate the growth and maintenance of all tissues through bidirectional signaling, mediated by the physical properties of the ECM along with its biochemical composition. Many cell culture models, including ex vivo organoids, have improved our understanding of ECM development and homeostasis; however, they do not replicate the complex microenvironment of the native ECM across individual tissue types. This Special Issue aims to explore current efforts used to bridge this gap in knowledge across multiple disciplines, including cell biology, genomics, biomechanical and biophysical characterisation, 3D cell culture, and bioengineered smart materials.

Dr. Larisa M. Haupt
Dr. Amaia Cipitria
Guest Editors

Manuscript Submission Information

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Keywords

  • extracellular matrix
  • biomechanical and biophysical characterisation
  • organoids
  • 3D culture
  • smart matrices
  • humanized models
  • proteoglycans

Published Papers (2 papers)

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Research

14 pages, 3595 KiB  
Article
Evaluation of Human-Induced Pluripotent Stem Cells Derived from a Patient with Schwartz–Jampel Syndrome Revealed Distinct Hyperexcitability in the Skeletal Muscles
by Yuri Yamashita, Satoshi Nakada, Kyoko Nakamura, Hidetoshi Sakurai, Kinji Ohno, Tomohide Goto, Yo Mabuchi, Chihiro Akazawa, Nobutaka Hattori and Eri Arikawa-Hirasawa
Biomedicines 2023, 11(3), 814; https://doi.org/10.3390/biomedicines11030814 - 7 Mar 2023
Cited by 2 | Viewed by 1466
Abstract
Schwartz–Jampel syndrome (SJS) is an autosomal recessive disorder caused by loss-of-function mutations in heparan sulfate proteoglycan 2 (HSPG2), which encodes the core basement membrane protein perlecan. Myotonia is a major criterion for the diagnosis of SJS; however, its evaluation is based [...] Read more.
Schwartz–Jampel syndrome (SJS) is an autosomal recessive disorder caused by loss-of-function mutations in heparan sulfate proteoglycan 2 (HSPG2), which encodes the core basement membrane protein perlecan. Myotonia is a major criterion for the diagnosis of SJS; however, its evaluation is based solely on physical examination and can be challenging in neonates and young children. Furthermore, the pathomechanism underlying SJS-related myotonia is not fully understood, and effective treatments for SJS are limited. Here, we established a cellular model of SJS using patient-derived human-induced pluripotent stem cells. This model exhibited hyper-responsiveness to acetylcholine as a result of abnormalities in the perlecan molecule, which were confirmed via comparison of their calcium imaging with calcium imaging of satellite cells derived from Hspg2−/−-Tg mice, which exhibit myotonic symptoms similar to SJS symptoms. Therefore, our results confirm the utility of creating cellular models for investigating SJS and their application in evaluating myotonia in clinical cases, while also providing a useful tool for the future screening of SJS therapies. Full article
(This article belongs to the Special Issue Human Extracellular Matrix in Homeostasis and Pathology)
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12 pages, 1772 KiB  
Article
Bioactive Cell-Derived ECM Scaffold Forms a Unique Cellular Microenvironment for Lung Tissue Engineering
by Ali Doryab and Otmar Schmid
Biomedicines 2022, 10(8), 1791; https://doi.org/10.3390/biomedicines10081791 - 26 Jul 2022
Cited by 9 | Viewed by 2209
Abstract
Chronic lung diseases are one of the leading causes of death worldwide. Lung transplantation is currently the only causal therapeutic for lung diseases, which is restricted to end-stage disease and limited by low access to donor lungs. Lung tissue engineering (LTE) is a [...] Read more.
Chronic lung diseases are one of the leading causes of death worldwide. Lung transplantation is currently the only causal therapeutic for lung diseases, which is restricted to end-stage disease and limited by low access to donor lungs. Lung tissue engineering (LTE) is a promising approach to regenerating a replacement for at least a part of the damaged lung tissue. Currently, lung regeneration is limited to a simplified local level (e.g., alveolar–capillary barrier) due to the sophisticated and complex structure and physiology of the lung. Here, we introduce an extracellular matrix (ECM)-integrated scaffold using a cellularization–decellularization–recellularization technique. This ECM-integrated scaffold was developed on our artificial co-polymeric BETA (biphasic elastic thin for air–liquid interface cell culture conditions) scaffold, which were initially populated with human lung fibroblasts (IMR90 cell line), as the main generator of ECM proteins. Due to the interconnected porous structure of the thin (<5 µm) BETA scaffold, the cells can grow on and infiltrate into the scaffold and deposit their own ECM. After a mild decellularization procedure, the ECM proteins remained on the scaffold, which now closely mimicked the cellular microenvironment of pulmonary cells more realistically than the plain artificial scaffolds. We assessed several decellularization methods and found that 20 mM NH4OH and 0.1% Triton X100 with subsequent DNase treatment completely removed the fibroblasts (from the first cellularization) and maintains collagen I and IV as the key ECM proteins on the scaffold. We also showed the repopulation of the primary fibroblast from human (without chronic lung disease (non-CLD) donors) and human bronchial epithelial (16HBE14o) cells on the ECM-integrated BETA scaffold. With this technique, we developed a biomimetic scaffold that can mimic both the physico-mechanical properties and the native microenvironment of the lung ECM. The results indicate the potential of the presented bioactive scaffold for LTE application. Full article
(This article belongs to the Special Issue Human Extracellular Matrix in Homeostasis and Pathology)
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