Pridopidine in the treatment of Huntington’s disease
-
Magdalena Jabłońska
,
Klaudyna Grzelakowska
Abstract
Huntington’s disease (HD) is a highly common inherited monogenic neurodegenerative disease, and the gene responsible for its development is located in the 4p16.3 chromosome. The product of that gene mutation is an abnormal huntingtin (Htt) protein that disrupts the neural conduction, thus leading to motor and cognitive disorders. The disease progresses to irreversible changes in the central nervous system (CNS). Although only a few drugs are available to symptomatic treatment, ‘dopamine stabilizers’ (as represented by the pridopidine) may be the new treatment options. The underlying causes of HD are dopaminergic conduction disorders. Initially, the disease is hyperkinetic (chorea) until it eventually reaches the hypokinetic phase. Studies confirmed a correlation between the amount of dopamine in the CNS and the stage of the disease. Pridopidine has the capacity to be a dopamine buffer, which could increase or decrease the dopamine content depending on the disease phase. A research carried out on animal models demonstrated the protective effect of pridopidine on nerve cells thanks to its ability to alter the cortical glutamatergic signaling through the N-methyl-D-aspartate (NMDA) receptors. Studies on dopamine stabilizers also reported that pridopidine has a 100-fold greater affinity for the sigma-1 receptor than for the D2 receptor. Disturbances in the activity of sigma-1 receptors occur in neurodegenerative diseases, including HD. Their interaction with pridopidine results in the neuroprotective effect, which is manifested as an increase in the plasticity of synaptic neurons and prevention of their atrophy within the striatum. To determine the effectiveness of pridopidine in the treatment of HD, large multicenter randomized studies such as HART, MermaiHD, and PRIDE-HD were carried out.
Conflict of interest statement: The authors declare no conflict of interest.
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Artikel in diesem Heft
- Frontmatter
- A complete overview of REEP1: old and new insights on its role in hereditary spastic paraplegia and neurodegeneration
- Brain energetics, mitochondria, and traumatic brain injury
- Amyloid-β, tau, and the cholinergic system in Alzheimer’s disease: seeking direction in a tangle of clues
- Gut dysbiosis and serotonin: intestinal 5-HT as a ubiquitous membrane permeability regulator in host tissues, organs, and the brain
- Assessment of the efficacy of passive cellular immunotherapy for glioma patients
- Pridopidine in the treatment of Huntington’s disease
- The involvement of the central nervous system in patients with COVID-19
Artikel in diesem Heft
- Frontmatter
- A complete overview of REEP1: old and new insights on its role in hereditary spastic paraplegia and neurodegeneration
- Brain energetics, mitochondria, and traumatic brain injury
- Amyloid-β, tau, and the cholinergic system in Alzheimer’s disease: seeking direction in a tangle of clues
- Gut dysbiosis and serotonin: intestinal 5-HT as a ubiquitous membrane permeability regulator in host tissues, organs, and the brain
- Assessment of the efficacy of passive cellular immunotherapy for glioma patients
- Pridopidine in the treatment of Huntington’s disease
- The involvement of the central nervous system in patients with COVID-19
