NAD+ improves neuromuscular development in a zebrafish model of FKRP-associated dystroglycanopathy

Skeletal Muscle
Erin C BaileyClarissa A Henry

Abstract

Secondary dystroglycanopathies are muscular dystrophies that result from mutations in genes that participate in Dystroglycan glycosylation. Glycosylation of Dystroglycan is essential for muscle fibers to adhere to the muscle extracellular matrix (myomatrix). Although the myomatrix is disrupted in a number of secondary dystroglycanopathies, it is unknown whether improving the myomatrix is beneficial for these conditions. We previously determined that either NAD+ supplementation or overexpression of Paxillin are sufficient to improve muscle structure and the myomatrix in a zebrafish model of primary dystroglycanopathy. Here, we investigate how these modulations affect neuromuscular phenotypes in zebrafish fukutin-related protein (fkrp) morphants modeling FKRP-associated secondary dystroglycanopathy. We found that NAD+ supplementation prior to muscle development improved muscle structure, myotendinous junction structure, and muscle function in fkrp morphants. However, Paxillin overexpression did not improve any of these parameters in fkrp morphants. As movement also requires neuromuscular junction formation, we examined early neuromuscular junction development in fkrp morphants. The length of neuromuscular junctions was disrupted ...Continue Reading

References

Sep 1, 1990·The Journal of Cell Biology·C E TurnerK Burridge
Aug 1, 1993·The Journal of Cell Biology·J M Ervasti, K P Campbell
Jul 1, 1995·Developmental Dynamics : an Official Publication of the American Association of Anatomists·C B KimmelT F Schilling
Feb 24, 1997·The Journal of Cell Biology·A E DeconinckK E Davies
May 20, 1998·Nature Genetics·Y K HayashiK Arahata
Aug 9, 2002·Developmental Biology·Nathan D Lawson, Brant M Weinstein
Jun 12, 2003·Neuromuscular Disorders : NMD·Vianney JayasinhaPaul T Martin
Nov 25, 2003·Journal of Medical Genetics·D Beltrán-Valero de BernabéH G Brunner
May 4, 2004·Journal of Medical Genetics·D Beltran-Valero de BernabéF Muntoni
Jun 24, 2004·Human Molecular Genetics·Kinga GawlikMadeleine Durbeej
Mar 24, 2006·Development·Fernanda BajancaSólveig Thorsteinsdóttir
Aug 26, 2006·Nature Clinical Practice. Neurology·Paul T Martin
Apr 18, 2007·Proceedings of the National Academy of Sciences of the United States of America·Thomas E HallPeter D Currie
Oct 5, 2007·PLoS Biology·Wolfram TempelCharles Brenner
Oct 17, 2007·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Kristen M KwanChi-Bin Chien
Jul 25, 2008·Journal of Cell Science·Nicholas O Deakin, Christopher E Turner
Apr 7, 2009·Neuromuscular Disorders : NMD·Ruth HerbstReginald E Bittner
May 7, 2009·Proceedings of the National Academy of Sciences of the United States of America·Jachinta E RooneyDean J Burkin
Oct 30, 2009·Proceedings of the National Academy of Sciences of the United States of America·Douglas P MillayJeffery D Molkentin
Dec 4, 2009·Human Molecular Genetics·Genri KawaharaLouis M Kunkel
Feb 15, 2011·Human Molecular Genetics·Yung-Yao LinDerek L Stemple
Mar 15, 2011·Current Opinion in Genetics & Development·Caroline GodfreyFrancesco Muntoni
Aug 10, 2011·Current Opinion in Neurology·Francesco MuntoniSusan C Brown
Sep 20, 2011·Human Molecular Genetics·Alasdair J WoodVolker Straub
Oct 31, 2012·PLoS Biology·Michelle F GoodyClarissa A Henry
Dec 12, 2012·Human Molecular Genetics·Jessica D GumersonDaniel E Michele
Feb 28, 2013·Genesis : the Journal of Genetics and Development·Joachim Berger, Peter D Currie

❮ Previous
Next ❯

Citations

Aug 4, 2020·Frontiers in Molecular Neuroscience·Lacramioara Fabian, James J Dowling
Dec 5, 2020·Trends in Cell Biology·Carolina Ortiz-CorderoRita C R Perlingeiro
Feb 10, 2021·Brain : a Journal of Neurology·Alistair T PagnamentaHenry Houlden
Nov 9, 2021·PLoS Biology·Francisco J Martínez-MorcilloVictoriano Mulero

❮ Previous
Next ❯

Methods Mentioned

BETA
glycosylation
transgenic
transgenics
glycosylate

Software Mentioned

Perl script
Adobe Photoshop
Perl
FIJI
MATLAB
LARGE
GraphPad PRISM
EmergenC

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.