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Publication Detail
Investigating muscle phenotype change with age in monogenic disease may provide new insights into the normal ageing process
  • Publication Type:
    Conference presentation
  • Authors:
    Suetterlin K, Mannikko R, Dick J, Bostock H, Grounds M, Sayer AA, Tan SV, Matthews E, Greensmith L, Hanna M
  • Date:
    04/04/2019
  • Name of Conference:
    12th UK Neuromuscular Translational Research Conference
  • Conference place:
    Newcastle
  • Conference start date:
    04/04/2019
  • Conference finish date:
    05/04/2019
  • Keywords:
    ageing, channelopathy, periodic paralysis, sarcopenia
  • Addresses:
    MRC Centre for Neuromuscular Diseases
    University College London
    Department of Neuromuscular Diseases
    London
    United Kingdom
Abstract
Background: It is often reported that the severity and frequency of acute attacks of paralysis in people with both hyperkalaemic and hypokalaemic periodic paralysis starts to reduce around age forty. Forty is also the age around which optimal motor performance in master athletes starts to decline and above which pathological changes e.g. up to 5 COX negative fibres on muscle biopsy, are accepted as within normal limits for age. Aims: 1. To determine if the reduction in periodic paralysis attack severity with age is conserved across species. 2. To investigate whether the reduction in periodic paralysis attack severity with age is a result of normal muscle ageing or ageing in the presence of single ion channel dysfunction. Methods: To minimise genetic and environmental confounders and standardise the paralytic attack, male hyperkalaemic periodic paralysis (HyperPP) mutant mice were compared to their wild-type brothers. All animals were housed in the same environment. A paralytic attack was induced on soleus muscle ex vivo in a tissue chamber maintained at 30 degrees Celsius using a solution containing 10mM potassium and 1.3mM Calcium as previously described. The results from young adult (13 to 26 weeks, n=5 WT, n=5 Hyper PP), middle-aged (43 to 70 weeks, n=4 WT, n= 5 Hyper PP) and old (>95 weeks, n=5 WT, n=8 Hyper PP) mice were compared. Results: Young and middle-aged soleus lost significantly more force than old soleus. This was true for both HyperPP (one-way ANOVA p=0.007) and wild-type (one-way ANOVA p=0.009) animals. This was not simply due to a reduction in absolute force as maintenance of force also occurred in old soleus that had greater or equal baseline tetanic force to young soleus. Tetanic force for 2 out of the 5 old wild-type soleus muscles was increased throughout the period of hyperkalaemia. This was never observed in young or middle-aged wild-type, or young, middle-aged or old HyperPP soleus. Conclusions: A reduction in periodic paralysis attack severity with age appears to be conserved across species. Our data suggests this phenomenon is the result of ‘normal’ muscle ageing rather than the chronic consequence of single ion channel dysfunction.
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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