The Contribution of Mast Cells to the Regulation of Elastic Fiber Tensometry in the Skin Dermis of Children with Marfan Syndrome
Abstract
:1. Introduction
2. Results
2.1. Normal Skin
2.2. Skin in Patients with Marfan Syndrome
3. Discussion
4. Materials and Methods
4.1. Case Selection
4.2. Tissue Probe Staining
4.3. Immunohistochemistry and Histochemistry
4.4. Image Acquisition
4.5. Quantitative Analysis
4.6. Controls
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
MFS | Marfan syndrome |
MCs | Mast cells |
ECM | Extracellular matrix |
TGF-β | Transforming growth factor-beta |
CPA3 | Carboxypeptidase A3 |
α-SMA | Alpha smooth muscle actin |
VEGF | Vascular endothelial growth factor |
IL | Interleukins |
bFGF | Basic fibroblast growth factor |
NGF | Nerve growth factor |
PDGF | Platelet-derived growth factor |
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Patients | Features of the Fibrous Component of the Skin Dermis | ||||
---|---|---|---|---|---|
Total Area of the Fibrous Component of the Skin Dermis (mm2) | Collagen Fibers | Elastic Fibers | |||
Integral Area (mm2) | Relative Content (%) | Integral Area (mm2) | Relative Content (%) | ||
Patient No. 1 * | 0.66 | 0.49 | 74.2 | 0.17 | 25.8 |
Patient No. 2 | 1.42 | 1.06 | 74.65 | 0.36 | 25.35 |
Patient No. 3 | 2.83 | 2.51 | 88.7 | 0.32 | 11.3 |
Patient No. 4 | 1.83 | 1.41 | 77 | 0.42 | 23.0 |
Patient No. 5 ** | 1.74 | 1.41 | 81 | 0.33 | 19.0 |
Patient No. 6 | 2.13 | 1.76 | 82.6 | 0.37 | 17.4 |
Patient No. 7 | 1.92 | 1.44 | 75 | 0.48 | 25 |
The norm No. 1 | 8.58 | 6.82 | 79.5 | 1.76 | 20.5 |
The norm No. 2 | 12.09 | 9.17 | 75.85 | 2.92 | 24.15 |
Antibodies | Host | Catalogue Nr. | Dilution | Source |
---|---|---|---|---|
Tryptase | Mouse monoclonal | #ab2378 | 1:3000 | AbCam, Cambridge, UK |
Carboxypeptidase A3 (CPA3) | Rabbit polyclonal | #ab251696 | 1:2000 | AbCam, UK |
Chymase | Mouse monoclonal | #ab2377 | 1:3000 | AbCam, UK |
TGF-β | Rabbit monoclonal | #ab215715 | 1:500 | AbCam, UK |
Alpha SMA | Mouse monoclonal | #ab7817 | 1:3000 | AbCam, UK |
Antibodies and Other Reagents | Source | Dilution | Label |
---|---|---|---|
Goat anti-mouse IgG Ab (#ab97035) | AbCam, UK | 1/300 | Cy3 |
Goat anti-rabbit IgG Ab (#ab150077) | AbCam, UK | 1/300 | Alexa Fluor 488 |
Secondary antibodies conjugated with horseradish peroxidase (Opal Polymer HRP Ms+Rb (#ARH1001EA)) | Akoya Biosciences, Marlborough, MA, USA | ready-to-use | Opal 480 Reagent Pack (#FP1500001KT) |
Secondary antibodies conjugated with horseradish peroxidase (Opal Polymer HRP Ms+Rb (#ARH1001EA)) | Akoya Biosciences, USA | ready-to-use | Opal 570 Reagent Pack (#FP1488001KT) |
Secondary antibodies conjugated with horseradish peroxidase (Opal Polymer HRP Ms+Rb (#ARH1001EA)) | Akoya Biosciences, USA | ready-to-use | Opal 690 Reagent Pack (#FP1497001KT) |
AmpliStain™ anti-Mouse 1-Step HRP (#AS-M1-HRP) | SDT GmbH, Baesweiler, Germany | ready-to-use | HRP |
AmpliStain™ anti-Rabbit 1-Step HRP (#AS-R1-HRP) | SDT GmbH, Baesweiler, Germany | ready-to-use | HRP |
4′,6-diamidino-2-phenylindole (DAPI, #D9542-5MG) | Sigma, Hamburg, Germany | 5 µg/mL | w/o |
VECTASHIELD® Mounting Medium (#H-1000) | Vector Laboratories, Burlingame, CA, USA | ready-to-use | w/o |
DAB Peroxidase Substrate Kit (#SK-4100) | Vector Laboratories, Burlingame, CA, USA | ready-to-use | DAB |
Mayer’s Hematoxylin (Biovitrum, #05-002) | ErgoProduction LLC, Saint Petersburg, Russia | ready-to-use | w/o |
Eosin Y 1% aqueous (Biovitrum, #05-010/S) | ErgoProduction LLC, Russia | ready-to-use | w/o |
Giemsa solution (Biovitrum, #21-023) | ErgoProduction LLC, Russia | ready-to-use | w/o |
Toluidine blue (Biovitrum, #07-002) | ErgoProduction LLC, Russia | ready-to-use | w/o |
Silver impregnation (Biovitrum, #21-026) | ErgoProduction LLC, Russia | ready-to-use | w/o |
Picro Mallory trichrome (Biovitrum, #21-036) | ErgoProduction LLC, Russia | ready-to-use | w/o |
Weigert for elastic fibers (Biovitrum, #21-030) | ErgoProduction LLC, Russia | ready-to-use | w/o |
Weigert–Van Gieson (Biovitrum, #21-020) | ErgoProduction LLC, Russia | ready-to-use | w/o |
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Atiakshin, D.; Nikolaeva, E.; Semyachkina, A.; Kostin, A.; Volodkin, A.; Morozov, S.; Ignatyuk, M.; Mikhaleva, L.; Demyashkin, G.; Elieh-Ali-Komi, D.; et al. The Contribution of Mast Cells to the Regulation of Elastic Fiber Tensometry in the Skin Dermis of Children with Marfan Syndrome. Int. J. Mol. Sci. 2024, 25, 9191. https://doi.org/10.3390/ijms25179191
Atiakshin D, Nikolaeva E, Semyachkina A, Kostin A, Volodkin A, Morozov S, Ignatyuk M, Mikhaleva L, Demyashkin G, Elieh-Ali-Komi D, et al. The Contribution of Mast Cells to the Regulation of Elastic Fiber Tensometry in the Skin Dermis of Children with Marfan Syndrome. International Journal of Molecular Sciences. 2024; 25(17):9191. https://doi.org/10.3390/ijms25179191
Chicago/Turabian StyleAtiakshin, Dmitrii, Ekaterina Nikolaeva, Alla Semyachkina, Andrey Kostin, Artem Volodkin, Sergey Morozov, Michael Ignatyuk, Liudmila Mikhaleva, Grigory Demyashkin, Daniel Elieh-Ali-Komi, and et al. 2024. "The Contribution of Mast Cells to the Regulation of Elastic Fiber Tensometry in the Skin Dermis of Children with Marfan Syndrome" International Journal of Molecular Sciences 25, no. 17: 9191. https://doi.org/10.3390/ijms25179191