Fig. 7.

Time-chart of HD phenotype development in TgHD minipig model. Results from present publication are colored in black; previously published (or unpublished) data are indicted in gray. Displayed biochemical mitochondrial parameters are dependent exclusively on HD disease (except proteins marked by #) and not on age or gender, so we propose these as biomarkers of HD development. ¹Baxa et al., 2013; ²Macakova et al., 2016; ³Krizova et al., 2017; ⁴Vidinska et al., 2018; ⁵Data described in present publication; ⁶Askeland et al., 2018b; ⁷See Movie 1; ⁸Taras Ardan, Z.E. et al., Institute of Animal Physiology and Genetics AS CR, Czech Republic, unpublished data. Enzymatic activity: COX, cytochrome c oxidase (RCCIV); CS, citrate synthase; NCCR, NADH-cytochrome c oxidase (RCCI-III); SQR/CS, ratio of succinate dehydrogenase (RCCII) to citrate synthase activity. Respiration parameters: GM, glutamate-malate (RCCI-dependent respiration); CII, RCCII-dependent respiration; CIV, RCCIV-dependent respiration; CI/CIV, ratio of RCCI- to RCCIV-dependent respiration. Proteins: SDH30, subunit of complex II; OSCP, oligomycin-sensitivity conferring protein subunit of ATPase (RCCV); OPA1, optic atrophy 1 protein; DRP1, dynamin-related protein 1.