A Role for Caveolin-3 in the Pathogenesis of Muscular Dystrophies

Caveolae are the cholesterol-rich small invaginations of the plasma membrane present in many cell types including adipocytes, endothelial cells, epithelial cells, fibroblasts, smooth muscles, skeletal muscles and cardiac muscles. They serve as specialized platforms for many signaling molecules and r...

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Published inInternational journal of molecular sciences Vol. 21; no. 22; p. 8736
Main Authors Pradhan, Bhola Shankar, Prószyński, Tomasz J
Format Journal Article
LanguageEnglish
Published Switzerland MDPI AG 19.11.2020
MDPI
Subjects
Adipocytes
Animals
Arrhythmias, Cardiac - genetics
Arrhythmias, Cardiac - metabolism
Arrhythmias, Cardiac - physiopathology
Arteriosclerosis
Atherosclerosis
Autophagy
Cardiac muscle
Cardiomegaly - genetics
Cardiomegaly - metabolism
Cardiomegaly - physiopathology
Cardiovascular diseases
Caveolae
Caveolae - metabolism
Caveolin
Caveolin 3 - chemistry
Caveolin 3 - genetics
Caveolin 3 - metabolism
Caveolin-3
Cell surface
Cholesterol
Diabetes mellitus
Dystrophin - genetics
Dystrophin - metabolism
endocytic pathway
Endocytosis
Endothelial cells
Energy metabolism
Epithelial cells
Fibroblasts
Gene Expression Regulation
Growth factors
Homeostasis
Humans
Invaginations
Kinases
Lipid metabolism
Lipids
Mechanotransduction
Mechanotransduction, Cellular
Membranes
Mice
Mice, Inbred mdx
Mitochondria
Muscle, Skeletal - metabolism
Muscle, Skeletal - physiopathology
Muscles
Muscular Dystrophies - genetics
Muscular Dystrophies - metabolism
Muscular Dystrophies - physiopathology
Muscular dystrophy
Mutation
Nerve Tissue Proteins - genetics
Nerve Tissue Proteins - metabolism
Neuromuscular Junction - genetics
Neuromuscular Junction - metabolism
Neuromuscular Junction - physiopathology
Oligomerization
Pathogenesis
Plasma
Potassium Channels, Tandem Pore Domain - genetics
Potassium Channels, Tandem Pore Domain - metabolism
Proteins
Receptors, Adrenergic, beta - genetics
Receptors, Adrenergic, beta - metabolism
Review
scaffolding domain
Signal transduction
Skeletal muscle
muscular dystrophy
scaffolding domain
Caveolin-3
cardiovascular diseases
dystroglycan
dystrophin
endocytic pathway
caveolae
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Abstract Caveolae are the cholesterol-rich small invaginations of the plasma membrane present in many cell types including adipocytes, endothelial cells, epithelial cells, fibroblasts, smooth muscles, skeletal muscles and cardiac muscles. They serve as specialized platforms for many signaling molecules and regulate important cellular processes like energy metabolism, lipid metabolism, mitochondria homeostasis, and mechano-transduction. Caveolae can be internalized together with associated cargo. The caveolae-dependent endocytic pathway plays a role in the withdrawal of many plasma membrane components that can be sent for degradation or recycled back to the cell surface. Caveolae are formed by oligomerization of caveolin proteins. Caveolin-3 is a muscle-specific isoform, whose malfunction is associated with several diseases including diabetes, cancer, atherosclerosis, and cardiovascular diseases. Mutations in Caveolin-3 are known to cause muscular dystrophies that are collectively called caveolinopathies. Altered expression of Caveolin-3 is also observed in Duchenne's muscular dystrophy, which is likely a part of the pathological process leading to muscle weakness. This review summarizes the major functions of Caveolin-3 in skeletal muscles and discusses its involvement in the pathology of muscular dystrophies.
AbstractList Caveolae are the cholesterol-rich small invaginations of the plasma membrane present in many cell types including adipocytes, endothelial cells, epithelial cells, fibroblasts, smooth muscles, skeletal muscles and cardiac muscles. They serve as specialized platforms for many signaling molecules and regulate important cellular processes like energy metabolism, lipid metabolism, mitochondria homeostasis, and mechano-transduction. Caveolae can be internalized together with associated cargo. The caveolae-dependent endocytic pathway plays a role in the withdrawal of many plasma membrane components that can be sent for degradation or recycled back to the cell surface. Caveolae are formed by oligomerization of caveolin proteins. Caveolin-3 is a muscle-specific isoform, whose malfunction is associated with several diseases including diabetes, cancer, atherosclerosis, and cardiovascular diseases. Mutations in Caveolin-3 are known to cause muscular dystrophies that are collectively called caveolinopathies. Altered expression of Caveolin-3 is also observed in Duchenne's muscular dystrophy, which is likely a part of the pathological process leading to muscle weakness. This review summarizes the major functions of Caveolin-3 in skeletal muscles and discusses its involvement in the pathology of muscular dystrophies.
Author Pradhan, Bhola Shankar
Prószyński, Tomasz J
AuthorAffiliation Łukasiewicz Research Network–PORT Polish Center for Technology Development, 147 Stabłowicka Street, 54-066 Wrocław, Poland; Bhola.Pradhan@port.lukasiewicz.gov.pl
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  organization: Łukasiewicz Research Network-PORT Polish Center for Technology Development, 147 Stabłowicka Street, 54-066 Wrocław, Poland
BackLink https://www.ncbi.nlm.nih.gov/pubmed/33228026$$D View this record in MEDLINE/PubMed
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Keywords muscular dystrophy
scaffolding domain
Caveolin-3
cardiovascular diseases
dystroglycan
dystrophin
endocytic pathway
caveolae
Language English
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SSID ssj0023259
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SecondaryResourceType review_article
Snippet Caveolae are the cholesterol-rich small invaginations of the plasma membrane present in many cell types including adipocytes, endothelial cells, epithelial...
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StartPage 8736
SubjectTerms Adipocytes
Animals
Arrhythmias, Cardiac - genetics
Arrhythmias, Cardiac - metabolism
Arrhythmias, Cardiac - physiopathology
Arteriosclerosis
Atherosclerosis
Autophagy
Cardiac muscle
Cardiomegaly - genetics
Cardiomegaly - metabolism
Cardiomegaly - physiopathology
Cardiovascular diseases
Caveolae
Caveolae - metabolism
Caveolin
Caveolin 3 - chemistry
Caveolin 3 - genetics
Caveolin 3 - metabolism
Caveolin-3
Cell surface
Cholesterol
Diabetes mellitus
Dystrophin - genetics
Dystrophin - metabolism
endocytic pathway
Endocytosis
Endothelial cells
Energy metabolism
Epithelial cells
Fibroblasts
Gene Expression Regulation
Growth factors
Homeostasis
Humans
Invaginations
Kinases
Lipid metabolism
Lipids
Mechanotransduction
Mechanotransduction, Cellular
Membranes
Mice
Mice, Inbred mdx
Mitochondria
Muscle, Skeletal - metabolism
Muscle, Skeletal - physiopathology
Muscles
Muscular Dystrophies - genetics
Muscular Dystrophies - metabolism
Muscular Dystrophies - physiopathology
Muscular dystrophy
Mutation
Nerve Tissue Proteins - genetics
Nerve Tissue Proteins - metabolism
Neuromuscular Junction - genetics
Neuromuscular Junction - metabolism
Neuromuscular Junction - physiopathology
Oligomerization
Pathogenesis
Plasma
Potassium Channels, Tandem Pore Domain - genetics
Potassium Channels, Tandem Pore Domain - metabolism
Proteins
Receptors, Adrenergic, beta - genetics
Receptors, Adrenergic, beta - metabolism
Review
scaffolding domain
Signal transduction
Skeletal muscle
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Title A Role for Caveolin-3 in the Pathogenesis of Muscular Dystrophies
URI https://www.ncbi.nlm.nih.gov/pubmed/33228026
https://www.proquest.com/docview/2463799229
https://search.proquest.com/docview/2464149648
https://pubmed.ncbi.nlm.nih.gov/PMC7699313
https://doaj.org/article/5be7b625a42c4a3297f8039b726bafac
Volume 21
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