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Related Concept Videos

Chemical Synapses01:26

Chemical Synapses

Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
GPCR Desensitization01:12

GPCR Desensitization

G protein-coupled receptor (GPCR) signaling plays a crucial role in cell functioning. GPCR desensitization is an equally essential process. It allows cells to respond to changing environments and regain sensitivity to new stimuli while preventing unnecessary stimulation when no longer needed. Prolonged exposure to stimuli leads to GPCR desensitization. It involves blocking the receptors from binding and activating additional G proteins. This inhibits activation of downstream effectors, thereby...
G-Protein Gated Ion Channels01:21

G-Protein Gated Ion Channels

GPCRs are primarily responsible for our sense of smell, taste, and vision.  The binding of a sensory stimulus activates GPCR to stimulate effector proteins, many of which are ion channels in the sensory organs. GPCRs modulate the opening and closing of the target ion channels either directly by binding them, or by releasing second messengers that activate these channels. As ions move across the membrane, the membrane potential is altered, which induces an appropriate response.
Sensory organs,...
GPCRs Regulate Adenylyl Cylase Activity01:09

GPCRs Regulate Adenylyl Cylase Activity

Some GPCRs transmit signals through adenylyl cyclase (AC), a transmembrane enzyme. AC helps synthesize second messenger cyclic adenosine monophosphate (cAMP). AC catalyzes cyclization reaction and converts ATP to cAMP by releasing a pyrophosphate. The pyrophosphate is further hydrolyzed to phosphate by the enzyme pyrophosphatase, which drives cAMP synthesis to completion. However, cAMP is rapidly degraded to 5′ AMP by the enzymes phosphodiesterase (PDE), preventing overstimulation of cells.
Two...
Ligand-Gated Ion Channel Receptor: Gating Mechanism01:30

Ligand-Gated Ion Channel Receptor: Gating Mechanism

Ligand-gated ion channels are transmembrane proteins that play a vital role in intercellular communication and functions of the nervous system. They allow the influx of ions across the membrane once the neurotransmitter binds, allowing the subsequent transmission of electrical excitation across the neurons. Other ligand-gated ion channels, like the γ-aminobutyric acid (GABA) receptor, permit anions like chloride into the cells on the binding of the GABA molecule. Their entry into the cell...
Cholinergic Receptors: Muscarinic01:25

Cholinergic Receptors: Muscarinic

The pharmacological actions of acetylcholine are elicited via its binding to two families of cholinergic receptors or cholinoceptors, namely, muscarinic and nicotinic receptors. Muscarinic receptors are G protein-coupled receptors and have five subtypes, M1–M5. All mAChR subtypes are activated by acetylcholine and blocked by the antagonist, atropine. 
The subtypes M1, M3, and M5 couple with the Gq subunit and activate the phospholipase C (PLC) activity, mobilizing intracellular Ca2+. Activation...

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Enhanced mGluR1 function causes motor deficits and region-specific Purkinje cell dysfunction.

Mohamed F Ibrahim1,2, Sevda Boyanova1,2, Yin Chun Cheng1,2

  • 1Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DU, UK.

Brain : a Journal of Neurology
|January 12, 2026
PubMed
Summary

Enhanced metabotropic glutamate receptor 1 (mGluR1) signaling causes spinocerebellar ataxia (SCA). This study

Keywords:
Grm1Purkinje cellataxiacerebellummGluR1selective vulnerability

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Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Background:

  • Spinocerebellar ataxias (SCAs) are inherited neurodegenerative disorders lacking effective treatments.
  • Metabotropic glutamate receptor 1 (mGluR1) signaling is implicated in SCAs, but its role in disease pathogenesis is debated.
  • Gain-of-function mutations in the metabotropic glutamate receptor 1 (Grm1) gene are linked to SCA44.

Purpose of the Study:

  • To investigate the role of enhanced mGluR1 signaling in SCA pathogenesis.
  • To develop and characterize a novel mouse model for studying SCA44.

Main Methods:

  • Generation of a mouse model with a gain-of-function mutation (p.Y792C) in the Grm1 gene.
  • Assessment of motor function, Purkinje cell (PC) activity, and synaptic innervation in Grm1 mutant mice.
  • Analysis of disease progression and regional specificity of pathology.

Main Results:

  • Grm1 mutant mice exhibit progressive motor deficits characteristic of SCA.
  • Overactive mGluR1 signaling leads to altered climbing fiber innervation and perturbed PC spontaneous activity.
  • Pathological changes are lobule- and disease-stage-specific, highlighting selective vulnerability of PC populations.

Conclusions:

  • Enhanced mGluR1 function is a direct cause of PC dysfunction and SCA pathology.
  • This mouse model provides insights into the mechanisms underlying SCA and selective neuronal vulnerability.
  • Findings clarify the role of mGluR1 signaling in neurodegeneration and suggest potential therapeutic targets.