Plectin is a linker protein that interacts with intermediate filaments and β4 integrin in hemidesmosomes of the epidermal basement membrane zone (BMZ). Type XVII collagen (COL17) has been suggested as another candidate plectin binding partner in hemidesmosomes. Here, we demonstrate that plectin-COL17 binding helps to maintain epidermal BMZ organization. We identified an epidermolysis bullosa (EB) simplex patient as having markedly diminished expression of plectin and COL17 in skin. The patient is compound heterozygous for sequence variants in the plectin gene (PLEC); one is a truncation and the other is a small in-frame deletion sequence variant. The in-frame deletion is located in the putative COL17-binding domain of plectin and abolishes the plectin-COL17 interaction in vitro. These results imply that disrupted interaction between plectin and COL17 is involved in the development of EB. Our study suggests that protein-protein binding defects may underlie EB in patients with unidentified disease-causing sequence variants.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/humu.23344 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Extracellular vesicles and citrullination signatures are novel biomarkers in sturgeon (Acipenser gueldenstaedtii) during chronic stress due to seasonal temperature challenge.
Fish Shellfish Immunol
November 2024
Pathobiology and Extracellular Vesicles Research Group, School of Life Sciences, University of Westminster, London W1W 6UW, UK. Electronic address:
Acipenser gueldenstaedtii is one of the most cultured sturgeon species worldwide and of considerable economic value for caviar production. There are though considerable challenges around chronic stress responses due to increased summer temperatures, impacting sturgeons' immune responses and their susceptibility to opportunistic infections. The identification of molecular and cellular pathways involved in stress responses may contribute to identifying novel biomarkers reflective of fish health status, crucial for successful sturgeon aquaculture.
View Article and Find Full Text PDFPlectin: Dual Participation in Tumor Progression.
Biomolecules
August 2024
College of Bioengineering, Chongqing University, Chongqing 400030, China.
The gene can encode a cytoskeletal linking protein, plectin, known for its interaction with three critical components of the cellular cytoskeleton: intermediate filaments, microtubules, and actin filaments. In recent years, more and more studies have reported that plectin is closely related to tumorigenesis and development, exhibiting both tumor-suppressive and tumor-promoting functions. Here, we first introduce the molecular structure and function of plectin, and then we summarize the current understanding of the crucial role of plectin in cancer progression.
View Article and Find Full Text PDFCandidate proteins interacting with cytoskeleton in cells from the basal airway epithelium .
Front Mol Biosci
July 2024
Department of Biological Sciences, Bowling Green State University, Bowling Green, OH, United States.
The cytoskeleton consists of actin, microtubules, septins, and intermediate filaments and, in most cells, is anchored to an extracellular matrix. Each cell has a unique arrangement of this network and readjusts it from time to time. To investigate the regulation of these reorganizations, we identified interactors from extracts of four cultured lines representing basal cells from the airway epithelium.
View Article and Find Full Text PDFCancer cell extravasation requires plectin-mediated delivery of MT1-MMP at invadopodia.
Br J Cancer
September 2024
Biological Sciences Platform, Sunnybrook Research Institute, Toronto, ON, Canada.
Article Synopsis
- The study investigates how MT1-MMP, a protease involved in cancer cell movement, is recruited to invadopodia in metastatic breast cancer cells, focusing on its interaction with a protein called plectin.
- Through techniques like mass spectrometry and siRNA, researchers found that plectin is crucial for forming invadopodia and facilitating the enzyme's delivery, significantly influencing cancer cell invasiveness.
- Targeting the MT1-MMP and plectin interaction might pave the way for new therapies aimed at reducing cancer metastasis.
Reconstitution of cytolinker-mediated crosstalk between actin and vimentin.
Eur J Cell Biol
June 2024
Department of Bionanoscience & Kavli Institute of Nanoscience, Delft University of Technology, 2629 HZ, Delft, the Netherlands. Electronic address:
Cell shape and motility are determined by the cytoskeleton, an interpenetrating network of actin filaments, microtubules, and intermediate filaments. The biophysical properties of each filament type individually have been studied extensively by cell-free reconstitution. By contrast, the interactions between the three cytoskeletal networks are relatively unexplored.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!