Referenced in: none

Automatically associated channels: Kir2.2 , Kir3.2 , Kir6.2

Title: Cell death in weaver mouse cerebellum.

Authors: Amy B Harkins, Aaron P Fox

Journal, date & volume: Cerebellum, 2002 Jul , 1, 201-6

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/12879981

Abstract
Mice with the weaver mutation exhibit an uneven weave to their gait, ataxia, mild locomotor hyperactivity and, occasionally, tonic-clonic seizures. A single amino acid mutation in a G-protein coupled, inwardly rectifying K+ channel, GIRK2, gives rise to the symptoms seen in the weaver mice. Two areas of the brain are primarily affected. Cerebellar granule cell neurons die soon after birth and dopaminergic neurons are severely depleted in the substantia nigra. In this article we review recent studies of wild-type and mutant GIRK channels found in native cells or introduced into expression systems. We also review two models that explain some of the details leading to the neuronal cell death observed in weaver mice.