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Lessons from cavin-1 deficiency

delete2020-01-10
delete22
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OA
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Libin Liu *
DOI:10.1042/BST20190380delete
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摘要

摘要

En 中文
Caveolae have been implicated in a wide range of critical physiological functions. In the past decade, the dominant role of cavin-1 in caveolae formation has been established, and it has been recognized as another master regulator for caveolae biology. Human patients with cavin-1 mutations develop lipodystrophy and muscular dystrophy and have some major pathological dysfunctions in fat tissue, skeleton muscle, heart, lung and other organs. Cavin-1 deficiency animal models consistently show similar phenotypes. However, the underlying molecular mechanisms remain to be elucidated. Recent studies have suggested many possible pathways, including mechanosensing, stress response, signal transduction, exosome secretion, and potential functions in the nucleus. Many excellent and comprehensive review articles already exist on the topics of caveolae structure formation, caveolins, and their pathophysiological functions. We will focus on recent studies using cavin-1 deficiency models, to summarize the pathophysiological changes in adipose, muscle, and other organs, followed by a summary of mechanistic studies about the roles of cavin-1, which includes caveolae formation, ribosomal RNA transcription, mechanical sensing, stress response, and exosome secretion. Further studies may help to elucidate the exact underlying molecular mechanism to explain the pathological changes observed in cavin-1 deficient human patients and animal models, so potential new therapeutic strategies can be developed.
Keyword:
TRANSCRIPT RELEASE FACTOR
CONGENITAL GENERALIZED LIPODYSTROPHY
POLYMERASE-I
FACTOR PTRF
CELLULAR SENESCENCE
MUSCULAR-DYSTROPHY
LIPID TRAFFICKING
NULL MICE
CAVEOLAE
PROTEIN

期刊

B
Biochemical Society Transactions
IF:
4.3
论文数:
7.8K
被引数:
1.4W

机构

B
boston university
学者数:
3.8W
论文数: 3.2W
被引数: 67
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