Mitochondrial Dysfunction in Skeletal Muscle Pathologies

被引：71

作者：

Abrigo, Johanna ^{[1
,2
,3
]}

Simon, Felipe ^{[2
,4
]}

Cabrera, Daniel ^{[5
,6
]}

Vilos, Cristian ^{[3
,7
]}

Cabello-Verrugio, Claudio ^{[1
,2
,3
]}

机构：

[1] Univ Andres Bello, Fac Ciencias Vida, Dept Ciencias Biol, Lab Muscle Pathol Fragil & Aging, Santiago 8370186, Chile

[2] Millennium Inst Immunol & Immunotherapy, Santiago, Chile

[3] Univ Santiago Chile, Ctr Dev Nanosci & Nanotechnol CEDENNA, Santiago, Chile

[4] Univ Andres Bello, Fac Ciencias Vida, Dept Ciencias Biol, Lab Integrat Physiopathol, Santiago, Chile

[5] Pontificia Univ Catolica Chile, Fac Med, Dept Gastroenterol, Santiago, Chile

[6] Univ Bernardo O Higgins, Fac Salud, Dept Ciencias Quim & Biol, Santiago, Chile

[7] Univ Talca, Sch Med, Ctr Med Res, Lab Nanomed & Targeted Delivery, Talca, Chile

来源：

CURRENT PROTEIN & PEPTIDE SCIENCE | 2019年 / 20卷 / 06期

基金：

欧盟地平线“2020”;

关键词：

Mitochondria; ROS; skeletal muscle; atrophy; dystrophy; ATP production; UBIQUITIN-PROTEASOME SYSTEM; DNA-DELETION MUTATIONS; DUCHENNE MUSCULAR-DYSTROPHY; PROTEIN-DEGRADATION; OXIDATIVE STRESS; HUNTINGTONS-DISEASE; INSULIN-RESISTANCE; PERMEABILITY TRANSITION; LACTATE PRODUCTION; MOLECULAR-BASIS;

D O I：

10.2174/1389203720666190402100902

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Several molecular mechanisms are involved in the regulation of skeletal Muscle function. Among them, mitochondrial activity can be identified. The mitochondria is an important and essential organelle in the skeletal muscle that is involved in metabolic regulation and ATP production, which are two key elements of muscle contractibility and plasticity. Thus, in this review, we present the critical and recent antecedents regarding the mechanisms through which mitochondrial dysfunction can be involved in the generation and development of skeletal muscle pathologies, its contribution to detrimental functioning in skeletal muscle and its crosstalk with other typical signaling pathways related to muscle diseases. In addition, an update on the development of new strategies with therapeutic potential to inhibit the deleterious impact of mitochondrial dysfunction in skeletal muscle is discussed.

引用

页码：536 / 546

页数：11

共 176 条

[31] Transcriptional repression of PGC-α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration [J].