Single Muscle Fiber Proteomics in Mitochondrial Disorders Highlights Fiber Type-Specific Adaptations to Respiratory Chain Defects

2018 ◽  
Author(s):  
Marta Murgia ◽  
Jing Tan ◽  
Philipp E. Geyer ◽  
Sophia Doll ◽  
Matthias Mann ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Muscle Fiber ◽  
Fiber Type ◽  
Mitochondrial Disorders ◽  
Single Muscle ◽  
Single Muscle Fiber ◽  
Chain Defects ◽  
Respiratory Chain Defects

scholarly journals Single Muscle Fiber Proteomics Reveals Fiber-Type-Specific Features of Human Muscle Aging

Cell Reports ◽  
2017 ◽  
Vol 19 (11) ◽  
pp. 2396-2409 ◽  
Author(s):  
Marta Murgia ◽  
Luana Toniolo ◽  
Nagarjuna Nagaraj ◽  
Stefano Ciciliot ◽  
Vincenzo Vindigni ◽  
...  
Keyword(s):  
Muscle Fiber ◽  
Fiber Type ◽  
Human Muscle ◽  
Single Muscle ◽  
Single Muscle Fiber ◽  
Muscle Aging

scholarly journals Sex- and fiber-type-related contractile properties in human single muscle fiber

2019 ◽  
Vol 15 (4) ◽  
pp. 537-545 ◽  
Author(s):  
Yunah Jeon ◽  
Junghwa Choi ◽  
Hee Jaeng Kim ◽  
Hojun Lee ◽  
Jae-Young Lim ◽  
...  
Keyword(s):  
Muscle Fiber ◽  
Fiber Type ◽  
Contractile Properties ◽  
Single Muscle ◽  
Single Muscle Fiber

scholarly journals PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

2009 ◽  
Vol 18 (10) ◽  
pp. 1805-1812 ◽  
Author(s):  
Sarika Srivastava ◽  
Francisca Diaz ◽  
Luisa Iommarini ◽  
Karine Aure ◽  
Anne Lombes ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Mitochondrial Disorders ◽  
Induced Expression ◽  
Chain Defects ◽  
Respiratory Chain Defects ◽  
In Cells

Fiber Type and Size as Sources of Variation in Human Single Muscle Fiber Passive Elasticity

2020 ◽  
Vol 142 (8) ◽  
Author(s):  
Alex M. Noonan ◽  
Derek P. Zwambag ◽  
Nicole Mazara ◽  
Erin Weersink ◽  
Geoffrey A. Power ◽  
...  
Keyword(s):  
Composite Materials ◽  
Muscle Fiber ◽  
Fiber Diameter ◽  
Fiber Type ◽  
Muscle Fibers ◽  
Cross Sectional Area ◽  
Single Muscle ◽  
Sectional Area ◽  
Cross Sectional ◽  
Single Muscle Fiber

Abstract Studies on single muscle fiber passive material properties often report relatively large variation in elastic modulus (or normalized stiffness), and it is not clear where this variation arises. This study was designed to determine if the stiffness, normalized to both fiber cross-sectional area and length, is inherently different between types 1 and 2 muscle fibers. Vastus lateralis fibers (n = 93), from ten young men, were mechanically tested using a cumulative stretch-relaxation protocol. SDS-PAGE classified fibers as types 1 or 2. While there was a difference in normalized stiffness between fiber types (p = 0.0019), an unexpected inverse relationship was found between fiber diameter and normalized stiffness (r = −0.64; p < 0.001). As fiber type and diameter are not independent, a one-way analysis of covariance (ANCOVA) including fiber diameter as a covariate was run; this eliminated the effect of fiber type on normalized stiffness (p = 0.1935). To further explore the relationship between fiber size and elastic properties, we tested whether stiffness was linearly related to fiber cross-sectional area, as would be expected for a homogenous material. Passive stiffness was not linearly related to fiber area (p < 0.001), which can occur if single muscle fibers are better represented as composite materials. The rule of mixtures for composite materials was used to explore whether the presence of a stiff perimeter-based fiber component could explain the observed results. The model (R2 = 0.38) predicted a perimeter-based normalized stiffness of 8800 ± 2600 kPa/μm, which is within the range of basement membrane moduli reported in the literature.

scholarly journals Extensive respiratory chain defects in inhibitory interneurones in patients with mitochondrial disease

2015 ◽  
Vol 42 (2) ◽  
pp. 180-193 ◽  
Author(s):  
Nichola Z. Lax ◽  
John Grady ◽  
Alex Laude ◽  
Felix Chan ◽  
Philippa D. Hepplewhite ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Mitochondrial Disease ◽  
Chain Defects ◽  
Respiratory Chain Defects

The biphasic morphology of voluntary and spontaneous single muscle fiber action potentials

1994 ◽  
Vol 17 (11) ◽  
pp. 1301-1307 ◽  
Author(s):  
Daniel Dumitru ◽  
John C. King ◽  
William van der Rijt ◽  
Dick F. Stegeman
Keyword(s):  
Muscle Fiber ◽  
Action Potentials ◽  
Single Muscle ◽  
Single Muscle Fiber ◽  
Fiber Action

Changes in the parameters of human single muscle fiber potentials with consecutive discharges

1982 ◽  
Vol 76 (1) ◽  
pp. 12-24 ◽  
Author(s):  
A. Gydikov ◽  
P. Gatev ◽  
G.V. Dimitrov ◽  
L. Gerilovsky
Keyword(s):  
Muscle Fiber ◽  
Single Muscle ◽  
Single Muscle Fiber

Mitochondrial Respiratory-Chain Defects Presenting as Nonspecific Features in Children

2000 ◽  
Vol 15 (7) ◽  
pp. 445-448 ◽  
Author(s):  
Chang Y. Tsao ◽  
Jerry R. Mendell ◽  
Warren D. Lo ◽  
Mark Luquette ◽  
Jerome Rusin
Keyword(s):  
Respiratory Chain ◽  
Mitochondrial Respiratory Chain ◽  
Chain Defects ◽  
Respiratory Chain Defects ◽  
Mitochondrial Respiratory

Single muscle fiber contractile properties in adults with muscular dystrophy treated with MYO-029

2009 ◽  
Vol 39 (1) ◽  
pp. 3-9 ◽  
Author(s):  
Lisa S. Krivickas ◽  
Ronan Walsh ◽  
Anthony A. Amato
Keyword(s):  
Muscular Dystrophy ◽  
Muscle Fiber ◽  
Contractile Properties ◽  
Single Muscle ◽  
Single Muscle Fiber

scholarly journals Time-Dependent and Tissue-Specific Accumulation of mtDNA and Respiratory Chain Defects in Chronic Doxorubicin Cardiomyopathy

Circulation ◽  
2003 ◽  
Vol 108 (19) ◽  
pp. 2423-2429 ◽  
Author(s):  
Dirk Lebrecht ◽  
Bernhard Setzer ◽  
Uwe-Peter Ketelsen ◽  
Jörg Haberstroh ◽  
Ulrich A. Walker
Keyword(s):  
Respiratory Chain ◽  
Time Dependent ◽  
Tissue Specific ◽  
Specific Accumulation ◽  
Chain Defects ◽  
Respiratory Chain Defects ◽  
Doxorubicin Cardiomyopathy
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