The sarcolemma in the Large(myd) mouse

Muscle & Nerve
Patrick W ReedRobert J Bloch

Abstract

In the Large(myd) mouse, dystroglycan is incompletely glycosylated and thus cannot bind its extracellular ligands, causing a muscular dystrophy that is usually lethal in early adulthood. We show that the Large(myd) mutation alters the composition and organization of the sarcolemma of fast-twitch skeletal muscle fibers in young adult mice. Costameres at the sarcolemma of the tibialis anterior muscle of Large(myd) mice contain reduced levels of several membrane cytoskeletal proteins, including dystrophin and beta-spectrin. In the quadriceps, longitudinally oriented costameric structures tend to become thickened and branched. More strikingly, proteins of the dystrophin complex present between costameres in controls are absent from Large(myd) muscles. We propose that the absence of the dystrophin complex from these regions destabilizes the sarcolemma of the Large(myd) mouse and thereby contributes to the severity of its muscular dystrophy. Thus, the positioning of sarcolemmal proteins may have a profound effect on the health of skeletal muscle.

References

May 1, 1976·The Journal of Heredity·P W LaneD D Myers
Feb 1, 1983·Proceedings of the National Academy of Sciences of the United States of America·J V PardoS W Craig
Jul 1, 1995·Journal of Neuropathology and Experimental Neurology·K D MathewsR Smith
Aug 1, 1993·The Journal of Cell Biology·J M Ervasti, K P Campbell
Apr 15, 1993·Proceedings of the National Academy of Sciences of the United States of America·B J PetrofH L Sweeney
Oct 1, 1996·Current Opinion in Cell Biology·M D Henry, K P Campbell
Oct 23, 1997·The Journal of Cell Biology·V StraubK P Campbell
Jan 20, 1999·Proceedings of the National Academy of Sciences of the United States of America·M PeyrardJ P Dumanski
Apr 13, 1999·Cell and Tissue Research·D J Burkin, S J Kaufman
Oct 26, 1999·Journal of Muscle Research and Cell Motility·M W Williams, R J Bloch
Jul 17, 2001·Brain Research. Developmental Brain Research·J A UrsittiR J Bloch
Mar 28, 2002·Physiological Reviews·Derek J BlakeKay E Davies
Jul 12, 2002·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Heather J SpenceSteven J Winder
Oct 24, 2002·Clinical Orthopaedics and Related Research·Robert J BlochJeanine A Ursitti
Jan 31, 2003·The Journal of Biological Chemistry·Daniel E Michele, Kevin P Campbell
Apr 29, 2003·Exercise and Sport Sciences Reviews·Robert J Bloch, Hugo Gonzalez-Serratos

❮ Previous
Next ❯

Citations

Sep 20, 2011·Human Molecular Genetics·Alasdair J WoodVolker Straub
Aug 28, 2012·The Journal of Clinical Investigation·Aaron M BeedleKevin P Campbell
Jan 27, 2006·Annals of Neurology·Patrick ReedRobert J Bloch
Jan 9, 2015·American Journal of Physiology. Cell Physiology·Karla P García-PelagioRobert J Bloch
Nov 14, 2019·American Journal of Physiology. Cell Physiology·Joaquin M MurielRobert J Bloch
Jan 11, 2020·Frontiers in Molecular Neuroscience·Peter D Yurchenco, Karen K McKee
Aug 13, 2021·Bioscience Reports·Erin BolandTom Van Agtmael

❮ 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.