Genetic determinants of disease severity in the myotonic dystrophy type 1 OPTIMISTIC cohort.
Sarah A CummingAura Cecilia Jimenez-MorenoKees OkkersenStephan WenningerFerroudja DaidjFiona HogarthRoberta LittlefordGráinne Siobhan GormanGuillaume BassezBenedikt SchoserHanns LochmüllerBaziel G M van EngelenDarren G Moncktonnull nullPublished in: Neurology (2019)
Careful characterization of the DMPK CTG repeat to define progenitor allele length and presence of variant repeats has increased utility in understanding clinical variability in a trial cohort and provides a genetic route for defining disease-specific outcome measures, and the basis of treatment response and stratification in DM1 trials.