The Role of Cullin-RING Ligases in Striated Muscle Development, Function, and Disease.
Nedd8
autophagy-lysosome system
cardiomyopathy
cullin-RING ligase (CRL)
muscular dystrophy
protein degradation
striated muscle development
striated muscle function
ubiquitin-proteasome system (UPS)
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
26 Oct 2020
26 Oct 2020
Historique:
received:
24
09
2020
revised:
11
10
2020
accepted:
13
10
2020
entrez:
29
10
2020
pubmed:
30
10
2020
medline:
4
3
2021
Statut:
epublish
Résumé
The well-orchestrated turnover of proteins in cross-striated muscles is one of the fundamental processes required for muscle cell function and survival. Dysfunction of the intricate protein degradation machinery is often associated with development of cardiac and skeletal muscle myopathies. Most muscle proteins are degraded by the ubiquitin-proteasome system (UPS). The UPS involves a number of enzymes, including E3-ligases, which tightly control which protein substrates are marked for degradation by the proteasome. Recent data reveal that E3-ligases of the cullin family play more diverse and crucial roles in cross striated muscles than previously anticipated. This review highlights some of the findings on the multifaceted functions of cullin-RING E3-ligases, their substrate adapters, muscle protein substrates, and regulatory proteins, such as the Cop9 signalosome, for the development of cross striated muscles, and their roles in the etiology of myopathies.
Identifiants
pubmed: 33114658
pii: ijms21217936
doi: 10.3390/ijms21217936
pmc: PMC7672578
pii:
doi:
Substances chimiques
Cullin Proteins
0
Muscle Proteins
0
COP9 Signalosome Complex
EC 3.4.19.12
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL152251
Pays : United States
Organisme : NHLBI NIH HHS
ID : HL128457
Pays : United States
Organisme : NIH HHS
ID : NS047101
Pays : United States
Organisme : NHLBI NIH HHS
ID : HL107744
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL128457
Pays : United States
Organisme : NIAMS NIH HHS
ID : P30 AR061303
Pays : United States
Organisme : NINDS NIH HHS
ID : P30 NS047101
Pays : United States
Organisme : NHLBI NIH HHS
ID : R00 HL107744
Pays : United States
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