GRID2 Chromosome 4

Glutamate ionotropic receptor delta type subunit 2
15 variants 15 Health Risk

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What This Gene Does
The protein encoded by this gene is a member of the family of ionotropic glutamate receptors which are the predominant excitatory neurotransmitter receptors in the mammalian brain. The encoded protein is a multi-pass membrane protein that is expressed selectively in cerebellar Purkinje cells. A point mutation in the mouse ortholog, associated with the phenotype named 'lurcher', in the heterozygous state leads to ataxia resulting from selective, cell-autonomous apoptosis of cerebellar Purkinje cells during postnatal development. Mice homozygous for this mutation die shortly after birth from massive loss of mid- and hindbrain neurons during late embryogenesis. This protein also plays a role in synapse organization between parallel fibers and Purkinje cells. Alternate splicing results in multiple transcript variants encoding distinct isoforms. Mutations in this gene cause cerebellar ataxia in humans. [provided by RefSeq, Apr 2014]
Gene Info
Gene Group
Glutamate ionotropic receptor delta type subunits
Locus Type
gene with protein product
Location
4q22.1-q22.2
Ensembl
ENSG00000152208
Associated Conditions (4)
Autosomal recessive spinocerebellar ataxia 18
GRID2-related disorder
Inborn genetic diseases
See cases
Key Variants
All Variants (15)
RSID Category Clinical Significance Conditions
RS144455304 Health Risk Conflicting classifications of pathogenicity Autosomal recessive spinocerebellar ataxia 18, GRID2-related disorder, Autosomal recessive spinocerebellar ataxia 18
RS146342092 Health Risk Conflicting classifications of pathogenicity GRID2-related disorder, Inborn genetic diseases, GRID2-related disorder
RS202203896 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS538573274 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS762100538 Health Risk Conflicting classifications of pathogenicity
RS1470494014 Health Risk Likely pathogenic
RS1560556892 Health Risk Likely pathogenic
RS1579319300 Health Risk Likely pathogenic Autosomal recessive spinocerebellar ataxia 18, Autosomal recessive spinocerebellar ataxia 18
RS2546697855 Health Risk Likely pathogenic
RS368143665 Health Risk Likely pathogenic Autosomal recessive spinocerebellar ataxia 18, Autosomal recessive spinocerebellar ataxia 18
RS1726914026 Health Risk Pathogenic Inborn genetic diseases, Inborn genetic diseases
RS1732687174 Health Risk Pathogenic Autosomal recessive spinocerebellar ataxia 18, Autosomal recessive spinocerebellar ataxia 18
RS750331613 Health Risk Pathogenic Autosomal recessive spinocerebellar ataxia 18, Autosomal recessive spinocerebellar ataxia 18
RS2546697844 Health Risk Pathogenic/Likely pathogenic See cases, Autosomal recessive spinocerebellar ataxia 18, See cases
RS2546697890 Health Risk Pathogenic/Likely pathogenic Inborn genetic diseases, Inborn genetic diseases
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