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Table 1 Ataxic mouse models relevant to this study

From: Computational analysis of calcium signaling and membrane electrophysiology in cerebellar Purkinje neurons associated with ataxia

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*

  1. P = Polyglutamine expansion; S = Supersensitive IP3R1; A = Association of mutant Ataxin with IP3R1 C-terminal; R = Reduced IP3R1 abundance; I = Increased calcium release.