Tetraspanin CD82 is necessary for muscle stem cell activation and supports dystrophic muscle function.


Journal

Skeletal muscle
ISSN: 2044-5040
Titre abrégé: Skelet Muscle
Pays: England
ID NLM: 101561193

Informations de publication

Date de publication:
27 11 2020
Historique:
received: 01 06 2020
accepted: 09 11 2020
entrez: 27 11 2020
pubmed: 28 11 2020
medline: 5 10 2021
Statut: epublish

Résumé

Tetraspanins are a family of proteins known to assemble protein complexes at the cell membrane. They are thought to play diverse cellular functions in tissues by modifying protein-binding partners, thus bringing complexity and diversity in their regulatory networks. Previously, we identified the tetraspanin KAI/CD82 as a prospective marker for human muscle stem cells. CD82 expression appeared decreased in human Duchenne muscular dystrophy (DMD) muscle, suggesting a functional link to muscular dystrophy, yet whether this decrease is a consequence of dystrophic pathology or a compensatory mechanism in an attempt to rescue muscle from degeneration is currently unknown. We studied the consequences of loss of CD82 expression in normal and dystrophic skeletal muscle and examined the dysregulation of downstream functions in mice aged up to 1 year. Expression of CD82 is important to sustain satellite cell activation, as in its absence there is decreased cell proliferation and less efficient repair of injured muscle. Loss of CD82 in dystrophic muscle leads to a worsened phenotype compared to control dystrophic mice, with decreased pulmonary function, myofiber size, and muscle strength. Mechanistically, decreased myofiber size in CD82 Basal CD82 expression is important to dystrophic muscle, as its loss leads to significantly weakened myofibers and impaired muscle function, accompanied by decreased satellite cell activity that is unable to protect and repair myofiber damage.

Sections du résumé

BACKGROUND
Tetraspanins are a family of proteins known to assemble protein complexes at the cell membrane. They are thought to play diverse cellular functions in tissues by modifying protein-binding partners, thus bringing complexity and diversity in their regulatory networks. Previously, we identified the tetraspanin KAI/CD82 as a prospective marker for human muscle stem cells. CD82 expression appeared decreased in human Duchenne muscular dystrophy (DMD) muscle, suggesting a functional link to muscular dystrophy, yet whether this decrease is a consequence of dystrophic pathology or a compensatory mechanism in an attempt to rescue muscle from degeneration is currently unknown.
METHODS
We studied the consequences of loss of CD82 expression in normal and dystrophic skeletal muscle and examined the dysregulation of downstream functions in mice aged up to 1 year.
RESULTS
Expression of CD82 is important to sustain satellite cell activation, as in its absence there is decreased cell proliferation and less efficient repair of injured muscle. Loss of CD82 in dystrophic muscle leads to a worsened phenotype compared to control dystrophic mice, with decreased pulmonary function, myofiber size, and muscle strength. Mechanistically, decreased myofiber size in CD82
CONCLUSION
Basal CD82 expression is important to dystrophic muscle, as its loss leads to significantly weakened myofibers and impaired muscle function, accompanied by decreased satellite cell activity that is unable to protect and repair myofiber damage.

Identifiants

pubmed: 33243288
doi: 10.1186/s13395-020-00252-3
pii: 10.1186/s13395-020-00252-3
pmc: PMC7693590
doi:

Substances chimiques

Cd82 antigen, mouse 0
Kangai-1 Protein 0
Proto-Oncogene Proteins c-akt EC 2.7.11.1
TOR Serine-Threonine Kinases EC 2.7.11.1
PTEN Phosphohydrolase EC 3.1.3.67

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

34

Subventions

Organisme : NIAMS NIH HHS
ID : R01 AR069582
Pays : United States
Organisme : NICHD NIH HHS
ID : U54 HD090255
Pays : United States

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Auteurs

Arielle Hall (A)

Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA, 02115, USA.

Tatiana Fontelonga (T)

Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA, 02115, USA.

Alec Wright (A)

Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA, 02115, USA.

Katlynn Bugda Gwilt (K)

Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA, 02115, USA.

Jeffrey Widrick (J)

Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA, 02115, USA.

Alessandra Pasut (A)

Laboratory of Angiogenesis and Vascular metabolism, Center for Cancer Biology, VIB and KU Leuven, 3000, Leuven, Belgium.

Francesco Villa (F)

F.M. Kirby Neurobiology Center, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Cynthia K Miranti (CK)

Department of Cellular and Molecular Medicine, University of Arizona College of Medicine, Tucson, AZ, 85724, USA.

Devin Gibbs (D)

Molecular Biology Institute, UCLA, Los Angeles, CA, 90095, USA.

Evan Jiang (E)

The University of Pennsylvania, College of Arts and Sciences, Philadelphia, PA, 19104, USA.

Hui Meng (H)

Department of Pathology and Laboratory Medicine and Neuroscience Research Center, Medical College of Wisconsin, Milwaukee, WI, 53226, USA.

Michael W Lawlor (MW)

Department of Pathology and Laboratory Medicine and Neuroscience Research Center, Medical College of Wisconsin, Milwaukee, WI, 53226, USA.

Emanuela Gussoni (E)

Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA, 02115, USA. Emanuela.gussoni@enders.tch.harvard.edu.
The Stem Cell Program at Boston Children's Hospital, Boston, MA, 02115, USA. Emanuela.gussoni@enders.tch.harvard.edu.

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Classifications MeSH