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Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism

Artikel i vetenskaplig tidskrift
Författare J. Blondelle
V. Marrocco
M. Clark
P. Desmond
S. Myers
J. Nguyen
M. Wright
S. Bremner
E. Pierantozzi
S. Ward
E. Esteve
V. Sorrentino
M. Ghassemian
Stefan Lange
Publicerad i Communications Biology
Volym 2
Publiceringsår 2019
Publicerad vid Wallenberglaboratoriet
Språk en
Länkar dx.doi.org/10.1038/s42003-019-0405-...
Ämnesord 3-m syndrome, m-band, flp recombinase, striated-muscle, short stature, filamin c, protein, gene, microtubule, interacts
Ämneskategorier Biokemi och molekylärbiologi


Biological roles of obscurin and its close homolog Obsl1 (obscurin-like 1) have been enigmatic. While obscurin is highly expressed in striated muscles, Obsl1 is found ubiquitously. Accordingly, obscurin mutations have been linked to myopathies, whereas mutations in Obsl1 result in 3M-growth syndrome. To further study unique and redundant functions of these closely related proteins, we generated and characterized Obsl1 knockouts. Global Obsl1 knockouts are embryonically lethal. In contrast, skeletal muscle-specific Obsl1 knockouts show a benign phenotype similar to obscurin knockouts. Only deletion of both proteins and removal of their functional redundancy revealed their roles for sarcolemmal stability and sarcoplasmic reticulum organization. To gain unbiased insights into changes to the muscle proteome, we analyzed tibialis anterior and soleus muscles by mass spectrometry, uncovering additional changes to the muscle metabolism. Our analyses suggest that all obscurin protein family members play functions for muscle membrane systems.

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