title: The cellular mRNA expression of GABA and glutamate receptors in spinal motor neurons of SOD1 mice.
abstract: ALS is a fatal neurodegenerative disorder characterized by a selective loss of upper motor neurons in the motor cortex and lower motor neurons in the brain stem and spinal cord. About 10% of ALS cases are familial, in 10-20% of these, mutations in the gene coding for superoxide dismutase 1 (SOD1) can be detected. Overexpression of mutated SOD1 in mice created animal models which clinically resemble ALS. Abnormalities in glutamatergic and GABAergic neurotransmission presumably contribute to the selective motor neuron damage in ALS. By in situ hybridization histochemistry (ISH), we investigated the spinal mRNA expression of the GABAA and AMPA type glutamate receptor subunits at different disease stages on spinal cord sections of mutant SOD1 mice and control animals overexpressing wild-type SOD1 aged 40, 80, 120 days and at disease end-stage, i.e. around 140 days) (n=5, respectively). We detected a slight but statistically significant decrease of the AMPA receptor subunits GluR3 and GluR4 only in end stage disease animals.
authors.affiliation: Department of Neurology, Medizinische Hochschule Hannover, D-30623, Hannover, Germany. sup2010@med.cornell.edu
authors.forename: S
authors.initials: S
authors.lastname: Petri
authors.forename: S
authors.initials: S
authors.lastname: Schmalbach
authors.forename: J
authors.initials: J
authors.lastname: Grosskreutz
authors.forename: K
authors.initials: K
authors.lastname: Krampfl
authors.forename: C
authors.initials: C
authors.lastname: Grothe
authors.forename: R
authors.initials: R
authors.lastname: Dengler
authors.forename: L
authors.initials: L
authors.lastname: Van Den Bosch
authors.forename: W
authors.initials: W
authors.lastname: Robberecht
authors.forename: J
authors.initials: J
authors.lastname: Bufler
mesh_terms: D000375:Aging / Q000378:metabolism; D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology / Q000235:genetics; D000818:Animals; D017931:DNA Primers; D003720:Densitometry; D005904:Glial Fibrillary Acidic Protein / Q000235:genetics / Q000378:metabolism; D005911:Gliosis / Q000235:genetics / Q000473:pathology; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000378:metabolism*; D009154:Mutation / Q000502:physiology; D009410:Nerve Degeneration / Q000473:pathology; D012333:RNA, Messenger / Q000096:biosynthesis*; D018091:Receptors, AMPA / Q000096:biosynthesis / Q000235:genetics; D018079:Receptors, GABA / Q000096:biosynthesis*; D011963:Receptors, GABA-A / Q000096:biosynthesis / Q000235:genetics; D017470:Receptors, Glutamate / Q000096:biosynthesis*; D013116:Spinal Cord / Q000166:cytology / Q000378:metabolism*; D013482:Superoxide Dismutase / Q000235:genetics* / Q000502:physiology*; D000072105:Superoxide Dismutase-1
chemical_list: D017931:DNA Primers; D005904:Glial Fibrillary Acidic Protein; D012333:RNA, Messenger; D018091:Receptors, AMPA; D018079:Receptors, GABA; D011963:Receptors, GABA-A; D017470:Receptors, Glutamate; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1