Hyperekplexia Phenotype of Glycine Receptor α1 Subunit Mutant Mice Identifies Zn2+ as an Essential Endogenous Modulator of Glycinergic Neurotransmission
Male
Neuroscience(all)
HUMDISEASE
Glycine
Efferent Pathways
MOLNEURO
Cell Line
Mice
Mice, Neurologic Mutants
03 medical and health sciences
Organ Culture Techniques
Receptors, Glycine
Animals
Humans
0303 health sciences
Chimera
Neural Inhibition
Mice, Mutant Strains
Mice, Inbred C57BL
Disease Models, Animal
Phenotype
SIGNALING
Dystonic Disorders
Mutation
Female
Brain Stem
DOI:
10.1016/j.neuron.2006.09.035
Publication Date:
2006-11-27T14:57:23Z
AUTHORS (8)
ABSTRACT
Zn2+ is thought to modulate neurotransmission by affecting currents mediated ligand-gated ion channels and transmitter reuptake Na+-dependent transporter systems. Here, we examined the in vivo relevance of neuromodulation producing knockin mice carrying mutation D80A glycine receptor (GlyR) α1 subunit gene (Glra1). This substitution selectively eliminates potentiating effect on GlyR currents. Mice homozygous for Glra1(D80A) develop a severe neuromotor phenotype postnatally that resembles forms human hyperekplexia (startle disease) caused mutations genes. In spinal neurons brainstem slices from mice, expression, synaptic localization, basal glycinergic transmission were normal; however, potentiation spontaneous was significantly impaired. Thus, due loss containing GlyRs, indicating essential proper functioning neurotransmission.
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CITATIONS (107)
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