Ataxia model | Mutated gene product | Protein name | Pathology | Compensation *Observed/**Suggested Therapeutic |
---|---|---|---|---|
 BK -/- | BK | Large conductance calcium-activated voltage-gated potassium channel | Altered Precision Timing | --- |
IP3R1 -/- | IP3R1 | Inositol triphosphate receptor 1 | No IP3R1 | -- |
IP3R1 +/- | IP3R1 | Inositol triphosphate receptor 1 | R | S** |
IP3R1 delta18/delta18 | IP3R1 | Inositol triphosphate receptor 1 | R | S** |
IP3R1 opt/opt | IP3R1 | Inositol triphosphate receptor 1 | R | Equivocal |
 SCA15 | IP3R1 | Inositol triphosphate receptor 1 | R | S** |
 SCA16 | IP3R1 | Inositol triphosphate receptor 1 | R | S** |
 PMCA -/- | PMCA | Plasma Membrane Calcium ATPase | No PCMA | R* |
 SCA1 | Ataxin1 | Ataxin-1 | P,A,I | R* |
 SCA2 | Ataxin2 | Ataxin-2 | P,A,S,I | R** |
 SCA3 | Ataxin3 | Ataxin-3 | P,A,S,I | R* |