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. Glycemic control and use of glucose-lowering medications in hospital-admitted type 2 diabetes patients over 80 years

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Lipidomic profiles, lipid trajectories and clinical biomarkers in female elite endurance athletes

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Describing the fecal metabolome in cryogenically collected samples from healthy participants

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  4. Author Correction: Characterisation and localisation of the endocannabinoid system components in the adult human testis

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  1. Absence of p.R50X Pygm read-through in McArdle disease cellular models

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. European muscle MRI study in limb girdle muscular dystrophy type R1/2A (LGMDR1/LGMD2A)

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Hydroxylated Long-Chain Acylcarnitines are Biomarkers of Mitochondrial 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