Spongy Degeneration with Cerebellar Ataxia

Gene involved
KCNJ10

Mutation tested
c.986T>C (SDCA1)

Transmission mode : Autosomal recessive

Publication : Mauri et al. (2017). A missense variant in KCNJ10 in Belgian Shepherd dogs affected by spongy degeneration with cerebellar ataxia (SDCA1).
Stee et al. (2022). Spinocerebellar ataxia in the Bouvier des Ardennes breed is caused by a KCNJ10 missense variant.

Gene involved
ATP1B2

Mutation tested
c.130_131ins227 (SDCA2)

Transmission mode : Autosomal recessive

Publication : Mauri et al. (2017). A SINE insertion in ATP1B2 in Belgian Shepherd dogs affected by spongy degeneration with cerebellar ataxia (SDCA2).