Progressive Early-Onset Cerebellar Ataxia
Body function: Muscle & Neurological
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Progressive early-onset cerebellar ataxia is a hereditary neurological disorder that primarily affects Finnish Hounds, though it has also been observed in Norrbottenspets. This condition is caused by a mutation in the SEL1L gene, which plays a role in the endoplasmic reticulum-associated protein degradation (ERAD) machinery. The mutation results in progressive degeneration of the cerebellum, a brain region crucial for motor control and coordination.
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