Table 1 Disease progression in different SBMA mouse models

From: Defective excitation-contraction coupling and mitochondrial respiration precede mitochondrial Ca2+ accumulation in spinobulbar muscular atrophy skeletal muscle

 

Presymptomatic

Disease onset

Advanced stage

Reference

AR100Q

Transgenic mice

4 weeks of age

8 weeks of age

12 weeks of age

5

AR113Q

Knock-in mice

12 weeks of age

24 weeks of age

48 weeks of age

7

iAR100Q

Conditional mice

4 weeks of age

7/8 weeks of age

12 weeks of age

5

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