Forskning
Udskriv Udskriv
Switch language
Region Hovedstaden - en del af Københavns Universitetshospital
Udgivet

Muscle contractility in spinobulbar muscular atrophy

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

  1. Body mass index in young men and risk of inflammatory bowel disease through adult life: A population-based Danish cohort study

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Leukocyte telomere length is associated with elevated plasma glucose and HbA1c in young healthy men independent of birth weight

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  1. Hydroxylated Long-Chain Acylcarnitines are Biomarkers of Mitochondrial Myopathy

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Dynamic soft tissue changes in the orbit after a blowout fracture

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Fat oxidation is impaired during exercise in lipin-1 deficiency

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  4. Impaired Fat Oxidation During Exercise in Long-Chain Acyl-CoA Dehydrogenase Deficiency Patients and Effect of IV-Glucose

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  5. Impairments in contractility and cytoskeletal organisation cause nuclear defects in nemaline myopathy

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

Vis graf over relationer

Spinobulbar muscular atrophy (SBMA) is caused by a trinucleotide repeat expansion in the androgen receptor gene on the X chromosome. There is a toxic effect of the mutant receptor on muscle and neurons resulting in muscle weakness and atrophy. The weakness can be explained by wasting due to loss of muscle cells, but it is unknown whether weakness also relates to poor muscle contractility of the remaining musculature. In this study, we investigated the muscle contractility in SBMA. We used stationary dynamometry and quantitative MRI to assess muscle strength and absolute and fat-free, cross-sectional areas. Specific muscle force (strength per cross-sectional area) and contractility (strength per fat-free cross-sectional area) were compared with healthy controls and their relation to walking distance and disease severity was investigated. Specific force was reduced by 14-49% in SBMA patients compared to healthy controls. Contractility was reduced by 22-39% in elbow flexion, knee extension, ankle dorsi- and plantarflexion in SBMA patients. The contractility decreased with increasing muscle fat content in muscles with affected contractility in SBMA. The decreased muscle contractility in SBMA may relate to motor neuron degeneration and changed fibre type distribution and muscle architecture.

OriginalsprogEngelsk
TidsskriftScientific Reports
Vol/bind9
Udgave nummer1
Sider (fra-til)4680
ISSN2045-2322
DOI
StatusUdgivet - 18 mar. 2019

ID: 58148202