Lurcher, nPIST, and autophagy.
Previous work has shown that neurodegeneration in the lurcher mouse is due to a mutation in the GluRdelta2 gene that results in a constitutively active glutamate receptor ion channel. Characterization of the cell death pathway in these animals reported by Yue et al. in this issue of Neuron provides important insight into the toxicity induced by the abundant transmitter glutamate. Through protein-protein interactions, the GluRdelta2(Lc) mutant channel activates autophagy.
Orr
Harry T
HT
Institute of Human Genetics, University of Minnesota, Minneapolis, MN 55455, USA.
D048868:Adaptor Proteins, Signal Transducing; D000818:Animals; D001343:Autophagy / Q000502:physiology*; D002352:Carrier Proteins / Q000737:chemistry / Q000502:physiology; D000077614:Golgi Matrix Proteins; D008565:Membrane Proteins; D026901:Membrane Transport Proteins; D051379:Mice; D008818:Mice, Neurologic Mutants / Q000502:physiology*; D017470:Receptors, Glutamate / Q000502:physiology
D048868:Adaptor Proteins, Signal Transducing; D002352:Carrier Proteins; C434484:GOPC protein, human; D000077614:Golgi Matrix Proteins; D008565:Membrane Proteins; D026901:Membrane Transport Proteins; D017470:Receptors, Glutamate; C107913:glutamate receptor delta 2