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Published on: May 25, 2011
[P/Q-type voltage-dependent calcium channels in neurological disease]
1Servicio de Neurología, Hospital Universitario Miguel Servet, Zaragoza. josegazulla@wanadoo.es
Neurologia (Barcelona, Spain)
|June 19, 2007
Summary
Voltage-dependent calcium channels (P/QCCs) are crucial for neuronal function. Disturbances in these channels cause neurological diseases, with mutations leading to decreased calcium conductance and potential new treatment avenues.
Area of Science:
- Neuroscience
- Molecular Biology
- Genetics
Context:
- Voltage-dependent calcium channels (VDCCs) are essential for cellular calcium influx.
- The alpha1A subunit (CACNA1A gene) forms the P/Q-type calcium channel (P/QCC), specific to neurons.
- Ancillary subunits modulate P/QCC activity, which is vital for neuronal plasticity, survival, and neurotransmission.
Purpose:
- To review the structure, modulation, and function of normal P/QCCs.
- To discuss neurological diseases associated with P/QCC dysfunction.
- To highlight the impact of mutated P/QCCs on calcium conductance.
Summary:
- P/QCCs, formed by the alpha1A subunit, are critical for neuronal function, particularly in the cerebellum and hippocampus.
- Mutations in P/QCCs are linked to various neurological disorders, including spinocerebellar ataxia, epilepsy, and migraine.
- Electrophysiological studies consistently show reduced calcium conductance in mutated P/QCCs compared to wild-type channels.
Impact:
- Understanding P/QCC structure and function is key to deciphering the mechanisms of associated neurological diseases.
- Investigating calcium channelopathies offers insights into how altered channel function leads to disease.
- This research may pave the way for novel therapeutic strategies for neurological conditions linked to P/QCC dysfunction.
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Generally, all voltage-gated ion channels have a 'voltage-sensing domain' that spans the lipid bilayer. The charged residues in the sensor move in response to the membrane potential changes that open the channel allowing ions movement. There are several types of...
Generally, all voltage-gated ion channels have a 'voltage-sensing domain' that spans the lipid bilayer. The charged residues in the sensor move in response to the membrane potential changes that open the channel allowing ions movement. There are several types of...
01:26Voltage-gated Ion Channels
Voltage-gated ion channels are transmembrane proteins that open and close in response to changes in the membrane potential. They are present on the membranes of all electrically excitable cells such as neurons, heart, and muscle cells.
Generally, all voltage-gated ion channels have a 'voltage-sensing domain' that spans the lipid bilayer. The charged residues in the sensor move in response to the membrane potential changes that open the channel allowing ions movement. There are several types of...
Generally, all voltage-gated ion channels have a 'voltage-sensing domain' that spans the lipid bilayer. The charged residues in the sensor move in response to the membrane potential changes that open the channel allowing ions movement. There are several types of...
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01:24Non-gated Ion Channels
Ion channels are specialized proteins on the plasma membrane that allow charged ions to pass down their electrochemical gradient. Their main function is to maintain the membrane potential which is critical for cell viability. These channels are either gated or non-gated and can transport more than a thousand ions within milliseconds for the cellular event to occur.
Compared to the gated ion channels, the non-gated channels, also known as leakage or passive channels, have no gating mechanism.
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