ABSTRACT Lurcher (_Lc_) is a spontaneous, semidominant mouse neurological mutation1. Heterozygous Lurcher mice (_Lc_/+) display ataxia as a result of a selective, cell-autonomous and

apoptotic death of cerebellar Purkinje cells during postnatal development2,3,4. Homozygous Lurcher mice (_Lc_/_Lc_) die shortly after birth because of a massive loss of mid- and hindbrain

neurons during late embryogenesis5. We have used positional cloning to identify the mutations responsible for neurodegeneration in two independent _Lc_ alleles as G-to-A transitions that

change a highly conserved alanine to a threonine residue in transmembrane domain III of the mouse δ2 glutamate receptor gene (GluRδ2). _Lc_/+ Purkinje cells have a very high membrane

neuronal death in Lurcher mice may provide a physiologically relevant model for excitotoxic cell death. Access through your institution Buy or subscribe This is a preview of subscription

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INDUCES PROGRESSIVE NIGROSTRIATAL PATHWAY DOPAMINERGIC DEGENERATION AND LYSOSOMAL ABNORMALITIES Article Open access 20 July 2024 _GRID1_/GLUD1 HOMOZYGOUS VARIANTS LINKED TO INTELLECTUAL

DISABILITY AND SPASTIC PARAPLEGIA IMPAIR MGLU1/5 RECEPTOR SIGNALING AND EXCITATORY SYNAPSES Article Open access 28 February 2024 LOSS OF ZINC-FINGER PROTEIN 212 LEADS TO PURKINJE CELL DEATH

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_Methods Neurosci._ 19, 261–279 (1994). Article  CAS  Google Scholar  Download references ACKNOWLEDGEMENTS We thank W. Hu for technical assistance; R. MacKinnon and J. Imredy for advice and

help with oocyte physiology experiments; colleagues of the Heintz and Linden laboratories for discussion and support; and C. Desplan, R. Duvoisin, J. Friedman, A. Hirano, A. J. Hudspeth, A.

S. Peterson, N. Segil and Z. P. Sun for critical reading of the manuscript. J.Z., W.J. and N.H. are supported by HHMI; P.L.D. is supported by NIH/NIGMS; D.J.L. is supported by PHS MH51106,

the McKnight Foundation, the Develbiss Fund, and the National Alliance for Research on Schizophrenia and Depression. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Laboratory of Molecular

Biology, Howard Hughes Medical Institute, The Rockefeller University, 1230 York Avenue, New York, 10021, New York, USA Jian Zuo, Philip L. De Jager, Weining Jiang & Nathaniel Heintz *

Department of Neuroscience, The Johns Hopkins University School of Medicine, 725 North Wolfe Street, Baltimore, 21205, Maryland, USA Kanji A. Takahashi & David J. Linden Authors * Jian

Zuo View author publications You can also search for this author inPubMed Google Scholar * Philip L. De Jager View author publications You can also search for this author inPubMed Google

Scholar * Kanji A. Takahashi View author publications You can also search for this author inPubMed Google Scholar * Weining Jiang View author publications You can also search for this author

inPubMed Google Scholar * David J. Linden View author publications You can also search for this author inPubMed Google Scholar * Nathaniel Heintz View author publications You can also

search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to Nathaniel Heintz. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE

Zuo, J., De Jager, P., Takahashi, K. _et al._ Neurodegeneration in Lurcher mice caused by mutation in δ2 glutamate receptor gene. _Nature_ 388, 769–773 (1997). https://doi.org/10.1038/42009

Download citation * Received: 10 March 1997 * Accepted: 02 June 1997 * Issue Date: 21 August 1997 * DOI: https://doi.org/10.1038/42009 SHARE THIS ARTICLE Anyone you share the following link

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