Differential expression of genes involved in the degeneration and regeneration pathways in mouse models for muscular dystrophies.

Neuromolecular Medicine
P C G Onofre-OliveiraM Vainzof

Abstract

The genetically determined muscular dystrophies are caused by mutations in genes coding for muscle proteins. Differences in the phenotypes are mainly the age of onset and velocity of progression. Muscle weakness is the consequence of myofiber degeneration due to an imbalance between successive cycles of degeneration/regeneration. While muscle fibers are lost, a replacement of the degraded muscle fibers by adipose and connective tissues occurs. Major investigation points are to elicit the involved pathophysiological mechanisms to elucidate how each mutation can lead to a specific degenerative process and how the regeneration is stimulated in each case. To answer these questions, we used four mouse models with different mutations causing muscular dystrophies, Dmd (mdx), SJL/J, Large (myd) and Lama2 (dy2J) /J, and compared the histological changes of regeneration and fibrosis to the expression of genes involved in those processes. For regeneration, the MyoD, Myf5 and myogenin genes related to the proliferation and differentiation of satellite cells were studied, while for degeneration, the TGF-β1 and Pro-collagen 1α2 genes, involved in the fibrotic cascade, were analyzed. The result suggests that TGF-β1 gene is activated in the dy...Continue Reading

References

Nov 1, 1991·Developmental Biology·J X DiMarioR C Strohman
Nov 1, 1990·Journal of Biochemistry·M Yoshida, E Ozawa
Feb 8, 1970·Life Sciences·H Meier, J L Southard
Feb 1, 1984·Proceedings of the National Academy of Sciences of the United States of America·G BulfieldK J Moore
Dec 1, 1982·Mechanisms of Ageing and Development·E Schultz, B H Lipton
Aug 1, 1993·The Journal of Cell Biology·J M Ervasti, K P Campbell
Jan 1, 1997·Muscle & Nerve·A H WellerN L Rosenberg
Apr 1, 1997·Current Opinion in Neurology·V Straub, K P Campbell
Apr 13, 1999·Cell and Tissue Research·B Brand-Saberi, B Christ
Jul 18, 2001·Journal of Applied Physiology·T J Hawke, D J Garry
Oct 23, 2001·Journal of Molecular Neuroscience : MN·M VainzofM Zatz
Nov 6, 2002·Biochimica Et Biophysica Acta·Prabhjit K Grewal, Jane E Hewitt
Jun 12, 2003·Physiological Genomics·Sean C GoetschDaniel J Garry
Nov 15, 2003·Reproductive Biology and Endocrinology : RB&E·Jennifer C J Chen, David J Goldhamer
Jan 13, 2004·Physiological Reviews·Sophie B P Chargé, Michael A Rudnicki
Mar 17, 2004·Current Opinion in Neurology·Francesco MuntoniSusan C Brown
Sep 25, 2004·Muscle & Nerve·Luc E GosselinDaniel A Martinez
Feb 1, 2007·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Telma L F GouveiaMariz Vainzof
May 9, 2007·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Neil D Theise
Sep 20, 2007·Journal of Animal Science·Z Yablonka-ReuveniG Shefer
Jan 19, 2008·Journal of Molecular Neuroscience : MN·Mariz VainzofLydia U Yamamoto
Sep 5, 2008·Current Opinion in Neurology·Michela GuglieriHanns Lochmüller
Nov 20, 2008·Muscle & Nerve·Paulo C GhediniCaden Souccar

❮ Previous
Next ❯

Citations

Apr 5, 2016·Stem Cells International·Camila F AlmeidaMariz Vainzof
Aug 12, 2015·Current Opinion in Neurology·Andy H Vo, Elizabeth M McNally
Nov 15, 2014·Pflügers Archiv : European journal of physiology·Martin SteinbergerHeinrich Brinkmeier
May 15, 2013·The International Journal of Biochemistry & Cell Biology·Jonghyun ShinAshok Kumar
Dec 18, 2014·Histochemistry and Cell Biology·Anne-K GüttschesMatthias Vorgerd
Jan 1, 2013·The American Journal of Pathology·Itai SpectorMark Pines
May 17, 2011·Neurobiology of Aging·Holly R ChinneryPaul G McMenamin
Jan 1, 2014·European Journal of Pediatrics·Pierluigi MarzuilloEgidio Barbi
Jun 16, 2017·Current Stem Cell Reports·Veronica PiniHelen C O'Neill
Apr 18, 2021·Human Molecular Genetics·Ramesh S YadavaMani S Mahadevan

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.