Leonit Kiriaev
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View article: <i>Mdx</i>mice dosed with antisense to CD49d and dystrophin exon skip morpholino; reduced force loss and affected gene expression pathways support ATL1102 combination therapy in DMD patients
<i>Mdx</i>mice dosed with antisense to CD49d and dystrophin exon skip morpholino; reduced force loss and affected gene expression pathways support ATL1102 combination therapy in DMD patients Open
Background VLA-4 is a disease progression biomarker in patients with Duchenne Muscular Dystrophy (DMD) including those treated with corticosteroids. ATL1102 an antisense oligonucleotide (ASO) inhibitor of human CD49d chain of adhesion mole…
View article: alpha-Actinin-3 deficiency protects from the effects of acute cold exposure through altered skeletal muscle Ca2+ and OXPHOS signaling.
alpha-Actinin-3 deficiency protects from the effects of acute cold exposure through altered skeletal muscle Ca2+ and OXPHOS signaling. Open
A nonsense polymorphism in the ACTN3 gene (R577X, rs1815739) results in the loss of the fast skeletal muscle fiber protein α-actinin-3 in an estimated 1.5 billion humans worldwide. Homozygosity for this common polymorphism (ACTN3 577XX) do…
View article: Modern Insights into Muscle Glycogen Phosphorylase Activity
Modern Insights into Muscle Glycogen Phosphorylase Activity Open
Recent identification of new human muscle glycogen phosphorylation sites has renewed interest in understanding human variations in the regulation of glycogen metabolism and glucose homeostasis. This paper presents a detailed method for the…
View article: Six weeks of N-acetylcysteine antioxidant in drinking water decreases pathological fiber branching in MDX mouse dystrophic fast-twitch skeletal muscle
Six weeks of N-acetylcysteine antioxidant in drinking water decreases pathological fiber branching in MDX mouse dystrophic fast-twitch skeletal muscle Open
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 trigger of skeletal muscle necrosis in the destructive dyst…
View article: Eccentric contraction-induced strength loss in dystrophin-deficient muscle: Preparations, protocols, and mechanisms
Eccentric contraction-induced strength loss in dystrophin-deficient muscle: Preparations, protocols, and mechanisms Open
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 by transient and reversible alterations to sarcolemmal exci…
View article: Absence of the Z-disc protein α-actinin-3 impairs the mechanical stability of Actn3KO mouse fast-twitch muscle fibres without altering their contractile properties or twitch kinetics
Absence of the Z-disc protein α-actinin-3 impairs the mechanical stability of Actn3KO mouse fast-twitch muscle fibres without altering their contractile properties or twitch kinetics Open
Background A common polymorphism (R577X) in the ACTN3 gene results in the complete absence of the Z-disc protein α-actinin-3 from fast-twitch muscle fibres in ~ 16% of the world’s population. This single gene polymorphism has been subject …
View article: Lifespan Analysis of Dystrophic mdx Fast-Twitch Muscle Morphology and Its Impact on Contractile Function
Lifespan Analysis of Dystrophic mdx Fast-Twitch Muscle Morphology and Its Impact on Contractile Function Open
Duchenne muscular dystrophy is caused by the absence of the protein dystrophin from skeletal muscle and is characterized by progressive cycles of necrosis/regeneration. Using the dystrophin deficient mdx mouse model, we studied the morphol…
View article: Absence of the Z-disc Protein α-actinin-3 Impairs the Mechanical Stability of Actn3KO Mouse Fast- twitch Muscle Fibres without Altering their Contractile Properties or Twitch Kinetics
Absence of the Z-disc Protein α-actinin-3 Impairs the Mechanical Stability of Actn3KO Mouse Fast- twitch Muscle Fibres without Altering their Contractile Properties or Twitch Kinetics Open
Background: A common polymorphism (R577X) in the ACTN3 gene results in complete absence of the Z-disc protein α-actinin-3 from fast-twitch muscle fibres in ~16% of the world’s population. This single gene polymorphism has been subject to s…
View article: Absence of the Z-disc protein α-actinin-3 impairs the mechanical stability of <i>Actn3KO</i> mouse fast-twitch muscle fibres without altering their contractile properties or twitch kinetics
Absence of the Z-disc protein α-actinin-3 impairs the mechanical stability of <i>Actn3KO</i> mouse fast-twitch muscle fibres without altering their contractile properties or twitch kinetics Open
Background A common polymorphism (R577X) in the ACTN3 gene results in complete absence of the Z-disc protein α-actinin-3 from fast-twitch muscle fibres in ~16% of the world’s population. This single gene polymorphism has been subject to st…
View article: Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged <i>mdx</i> dystrophic mice by reducing pathological fibre branching
Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged <i>mdx</i> dystrophic mice by reducing pathological fibre branching Open
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 fast-twitch skeletal muscle fibres. We have previously reporte…
View article: Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged <i>mdx</i> dystrophic mice by reducing pathological fibre branching
Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged <i>mdx</i> dystrophic mice by reducing pathological fibre branching Open
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 fast-twitch skeletal muscle fibres. We have previously reporte…
View article: Lifespan analysis of dystrophic <i>mdx</i> fast-twitch muscle morphology and its impact on contractile function
Lifespan analysis of dystrophic <i>mdx</i> fast-twitch muscle morphology and its impact on contractile function Open
Duchenne muscular dystrophy is caused by the absence of the protein dystrophin from skeletal muscle and is characterized by progressive cycles of necrosis/regeneration. Using the dystrophin deficient mdx mouse model we studied the morpholo…
View article: Minocycline Treatment Reduces Mass and Force Output From Fast-Twitch Mouse Muscles and Inhibits Myosin Production in C2C12 Myotubes
Minocycline Treatment Reduces Mass and Force Output From Fast-Twitch Mouse Muscles and Inhibits Myosin Production in C2C12 Myotubes Open
Minocycline, a tetracycline-class of antibiotic, has been tested with mixed effectiveness on neuromuscular disorders such as amyotrophic lateral sclerosis, autoimmune neuritis and muscular dystrophy. The independent effect of minocycline o…
View article: Isolated Extensor Digitorum Longus Muscles from Old mdx Dystrophic Mice Show Little Force Recovery 120 Minutes after Eccentric Damage
Isolated Extensor Digitorum Longus Muscles from Old mdx Dystrophic Mice Show Little Force Recovery 120 Minutes after Eccentric Damage Open
View article: Branched fibers from old fast-twitch dystrophic muscles are the sites of terminal damage in muscular dystrophy
Branched fibers from old fast-twitch dystrophic muscles are the sites of terminal damage in muscular dystrophy Open
A striking pathological feature of dystrophinopathies is the presence of morphologically abnormal branched skeletal muscle fibers. The deterioration of muscle contractile function in Duchenne muscular dystrophy is accompanied by both an in…