Association of dystrophin and an integral membrane glycoprotein

Nature
K P Campbell, S D Kahl

Abstract

Duchenne muscular dystrophy (DMD) is caused by a defective gene found on the X-chromosome. Dystrophin is encoded by the DMD gene and represents about 0.002% of total muscle protein. Immunochemical studies have shown that dystrophin is localized to the sarcolemma in normal muscle but is absent in muscle from DMD patients. Many features of the predicted primary structure of dystrophin are shared with membrane cytoskeletal proteins, but the precise function of dystrophin in muscle is unknown. Here we report the first isolation of dystrophin from digitonin-solubilized skeletal muscle membranes using wheat germ agglutinin (WGA)-Sepharose. We find that dystrophin is not a glycoprotein but binds to WGA-Sepharose because of its tight association with a WGA-binding glycoprotein. The association of dystrophin with this glycoprotein is disrupted by agents that dissociate cytoskeletal proteins from membranes. We conclude that dystrophin is linked to an integral membrane glycoprotein in the sarcolemma. Our results indicate that the function of dystrophin could be to link this glycoprotein to the underlying cytoskeleton and thus help either to preserve membrane stability or to keep the glycoprotein non-uniformly distributed in the sarcolemma.

References

Sep 1, 1979·Proceedings of the National Academy of Sciences of the United States of America·H TowbinJ Gordon
Oct 1, 1988·Trends in Neurosciences·K P CampbellA H Sharp

❮ Previous
Next ❯

Citations

Feb 19, 2000·Microscopy Research and Technique·S C WatkinsL Billington
Feb 19, 2000·Microscopy Research and Technique·A A HackE M McNally
Feb 19, 2000·Microscopy Research and Technique·D Hartigan-O'Connor, J S Chamberlain
Jan 1, 1996·Cell Motility and the Cytoskeleton·M JamesG E Morris
Jan 1, 1997·Developmental Dynamics : an Official Publication of the American Association of Anatomists·A J Barber, E Lieth
Jun 17, 1998·Developmental Dynamics : an Official Publication of the American Association of Anatomists·K Wertz, E M Füchtbauer
Jul 9, 1998·Muscle & Nerve·M A WineingerR T Abresch
Oct 31, 2000·Microscopy Research and Technique·K A McGowan, M P Marinkovich
Sep 26, 2000·Muscle & Nerve·R D Cohn, K P Campbell
Feb 17, 2001·Muscle & Nerve·R DraviamS C Watkins
Jul 1, 1993·Developmental Dynamics : an Official Publication of the American Association of Anatomists·J A AireyJ Talvenheimo
Apr 15, 1995·Microscopy Research and Technique·T KobayashiT Baba
Dec 1, 1995·Muscle & Nerve·E E Dupont-VersteegdenR J McCarter
Jan 1, 1994·Acta Neuropathologica·R MassaG Bernardi
Jan 1, 1993·Mammalian Genome : Official Journal of the International Mammalian Genome Society·U GangadharanS D Brown
Jun 1, 1991·Neurochemical Research·A D GurusingheM T Hearn
Dec 1, 1991·Journal of Neurocytology·P R Lyons, C R Slater
Jan 1, 1992·Journal of Inherited Metabolic Disease·R G Worton
Jan 1, 1992·Journal of Inherited Metabolic Disease·K M Bushby
Jan 1, 1992·Journal of Inherited Metabolic Disease·O F Hutter
Feb 1, 2007·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Telma L F GouveiaMariz Vainzof
Sep 14, 2006·Journal of Human Genetics·Motoi Kanagawa, Tatsushi Toda
May 7, 2010·Molecules and Cells·Sung Haeng Lee, Roberto Dominguez
May 31, 1991·Clinica Chimica Acta; International Journal of Clinical Chemistry·F GasnierO Gateau-Roesch

❮ 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.