Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy.

Orphanet Journal of Rare Diseases
Martin PeslVladimir Rotrekl

Abstract

We describe the association of Becker muscular dystrophy (BMD) derived heart failure with the impairment of tissue homeostasis and remodeling capabilities of the affected heart tissue. We report that BMD heart failure is associated with a significantly decreased number of cardiovascular progenitor cells, reduced cardiac fibroblast migration, and ex vivo survival. Becker muscular dystrophy belongs to a class of genetically inherited dystrophin deficiencies. It affects male patients and results in progressive skeletal muscle degeneration and dilated cardiomyopathy leading to heart failure. It is a relatively mild form of dystrophin deficiency, which allows patients to be on a heart transplant list. In this unique situation, the explanted heart is a rare opportunity to study the degenerative process of dystrophin-deficient cardiac tissue. Heart tissue was excised, dissociated, and analyzed. The fractional content of c-kit+/CD45- cardiovascular progenitor cells (CVPCs) and cardiac fibroblast migration were compared to control samples of atrial tissue. Control tissue was obtained from the hearts of healthy organ donor's during heart transplantation procedures. We report significantly decreased CVPCs (c-kit+/CD45-) throughout the hea...Continue Reading

References

Oct 9, 2004·Circulation Research·Elisa MessinaAlessandro Giacomello
Oct 9, 2008·The Canadian Journal of Cardiology·Josef Finsterer, Claudia Stöllberger
Nov 6, 2008·Journal of Cardiovascular Magnetic Resonance : Official Journal of the Society for Cardiovascular Magnetic Resonance·Ali YilmazUdo Sechtem
May 27, 2010·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Jon-Jon SantiagoIan M C Dixon
Jul 30, 2011·Skeletal Muscle·Christopher J MannPura Muñoz-Cánoves
Apr 20, 2012·Cell Transplantation·M CassanoM Sampaolesi
Aug 11, 2012·Journal of Cardiovascular Translational Research·Jacquelyn D Lajiness, Simon J Conway
Sep 5, 2012·Fibrogenesis & Tissue Repair·Dong FanZamaneh Kassiri
Jul 5, 2013·PloS One·Katrina K Treloar, Matthew J Simpson
Dec 3, 2013·Journal of the American College of Cardiology·Brian D Hoit
Mar 22, 2014·Circulation Research·Milena B FurtadoNadia A Rosenthal
Apr 12, 2014·Clinical Research in Cardiology : Official Journal of the German Cardiac Society·Sybilla MatuszczakStanisław Szala
May 31, 2014·BioMed Research International·Yacine KharrazPura Muñoz-Cánoves
Apr 17, 2015·Comprehensive Physiology·David DostalTroy A Baudino
Nov 17, 2015·Nature Medicine·Nicolas A DumontMichael A Rudnicki
Feb 4, 2016·Circulation Research·Conrad P HodgkinsonVictor J Dzau
Feb 4, 2016·Development·Milena B FurtadoNadia A Rosenthal
Jun 25, 2016·Circulation Research·Andrey Kazakov, Ulrich Laufs
Jun 30, 2016·World Journal of Cardiology·Rady HoPaul Mather
Jun 6, 2017·Intractable & Rare Diseases Research·Andrea Antonio PapaLuisa Politano
Aug 5, 2017·Frontiers in Cardiovascular Medicine·Yin Yee LeongJun Jie Tan
Sep 20, 2017·Transplantation·Ondrej ViklickyRobert Lischke
Jan 27, 2018·Matrix Biology : Journal of the International Society for Matrix Biology·Li LiWei Kong
Feb 18, 2018·The Journal of Thoracic and Cardiovascular Surgery·Megumi Mathison, Todd K Rosengart
Mar 3, 2018·Circulation Research·Hui ZhangBin Zhou
Sep 4, 2018·Frontiers in Cardiovascular Medicine·Virpi Talman, Riikka Kivelä
Apr 2, 2019·European Heart Journal·Darryl R Davis

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Citations

Jul 14, 2020·Frontiers in Bioengineering and Biotechnology·Sarka JelinkovaVladimir Rotrekl
Jun 3, 2021·International Journal of Molecular Sciences·Sarka JelinkovaAlbano C Meli
Jun 26, 2021·Pflügers Archiv : European journal of physiology·Barbora SvobodovaVladimir Rotrekl

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Methods Mentioned

BETA
biopsy
FACS

Software Mentioned

ImageJ
FlowJo
GraphPad
Prism
QuickPhoto Camera

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