Histological methods to assess skeletal muscle degeneration and regeneration in Duchenne muscular dystrophy

N Dubuisson, R Versele, C Planchon… - International journal of …, 2022 - mdpi.com
Duchenne muscular dystrophy (DMD) is a progressive disease caused by the loss of
function of the protein dystrophin. This protein contributes to the stabilisation of striated cells …

Odd skipped-related 1 controls the pro-regenerative response of fibro-adipogenic progenitors

G Kotsaris, TH Qazi, CH Bucher, H Zahid… - npj Regenerative …, 2023 - nature.com
Skeletal muscle regeneration requires the coordinated interplay of diverse tissue-resident-
and infiltrating cells. Fibro-adipogenic progenitors (FAPs) are an interstitial cell population …

Six weeks of N-acetylcysteine antioxidant in drinking water decreases pathological fiber branching in MDX mouse dystrophic fast-twitch skeletal muscle

A Redwan, L Kiriaev, S Kueh, JW Morley… - Frontiers in …, 2023 - frontiersin.org
Introduction: It has been proposed that an increased susceptivity to oxidative stress caused
by the absence of the protein dystrophin from the inner surface of the sarcolemma is a …

Hyperglycemia negatively affects IPSC-Derived myoblast proliferation and skeletal muscle regeneration and function

A Badu-Mensah, P Valinski, H Parsaud, JJ Hickman… - Cells, 2022 - mdpi.com
Diabetic myopathy is a co-morbidity diagnosed in most diabetes mellitus patients, yet its
pathogenesis is still understudied, which hinders the development of effective therapies …

Decoding microtubule detyrosination: enzyme families, structures, and functional implications

J Bak, TR Brummelkamp, A Perrakis - FEBS letters, 2024 - Wiley Online Library
Microtubules are a major component of the cytoskeleton and can accumulate a plethora of
modifications. The microtubule detyrosination cycle is one of these modifications; it involves …

Detyrosinated microtubule arrays drive myofibrillar malformations in mdx muscle fibers

AD Harriot, T Altair Morris, C Vanegas… - Frontiers in Cell and …, 2023 - frontiersin.org
Altered myofibrillar structure is a consequence of dystrophic pathology that impairs skeletal
muscle contractile function and increases susceptibility to contraction injury. In murine …

[HTML][HTML] Multimodal three-dimensional characterization of murine skeletal muscle micro-scale elasticity, structure, and composition: Impact of dysferlinopathy …

EM Lloyd, MS Hepburn, J Li, A Mowla, JH Jeong… - Journal of the …, 2024 - Elsevier
Skeletal muscle tissue function is governed by the mechanical properties and organization
of its components, including myofibers, extracellular matrix, and adipose tissue, which can …

Eccentric contraction-induced strength loss in dystrophin-deficient muscle: Preparations, protocols, and mechanisms

L Kiriaev, CW Baumann, A Lindsay - Journal of General Physiology, 2023 - rupress.org
The absence of dystrophin hypersensitizes skeletal muscle of lower and higher vertebrates
to eccentric contraction (ECC)-induced strength loss. Loss of strength can be accompanied …

Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged mdx dystrophic mice by reducing pathological fibre …

L Kiriaev, PJ Houweling, KN North… - Human Molecular …, 2022 - academic.oup.com
The common null polymorphism (R577X) in the ACTN3 gene is present in over 1.5 billion
people worldwide and results in the absence of the protein α-actinin-3 from the Z-discs of …

Myofibrillar malformations that arise in mdx muscle fibers are driven by detyrosinated microtubules

A Harriot, T Altair-Morris, C Venegas, J Kallenbach… - bioRxiv, 2023 - biorxiv.org
In Duchenne muscular dystrophy (DMD), alterations in the myofibrillar structure of skeletal
muscle fibers that impair contractile function and increase injury susceptibility arise as a …