ArticleJournal of molecular neuroscience : MN2021
ClC-2-like Chloride Current Alterations in a Cell Model of Spinal and Bulbar Muscular Atrophy, a Polyglutamine Disease.
Article in Journal of molecular neuroscience : MN, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 19 citations in OpenAlex.
- Unveiling the effect of curcumin on ion channels of SBMA motoneuron-derived cells and human IPSC-derived neurons: initial electrophysiological findings.European biophysics journal : EBJ · 2026Article
- Chloride Homeostasis in Neuronal Disorders: Bridging Measurement to Therapy.Life (Basel, Switzerland) · 2025Review
- A Theoretical and Practical Analysis of Membrane Protein Genes Altered in Neutrophils in Parkinson's Disease.Current issues in molecular biology · 2025Article
- Exploring the Role of Neuropeptide PACAP in Cytoskeletal Function Using Spectroscopic Methods.International journal of molecular sciences · 2024Article
- Nucleoporin Nup358 Downregulation Tunes the Neuronal Excitability in Mouse Cortical Neurons.Life (Basel, Switzerland) · 2023Article
- Female reproductive functions of the neuropeptide PACAP.Frontiers in endocrinology · 2022Review
- The Protective Effects of Endogenous PACAP in Oxygen-Induced Retinopathy.Journal of molecular neuroscience : MN · 2021Article
- Protective Effects of PACAP in a Rat Model of Diabetic Neuropathy.International journal of molecular sciences · 2021Article
- PACAP and NAP: Effect of Two Functionally Related Peptides in Diabetic Retinopathy.Journal of molecular neuroscience : MN · 2021Review
- Clenbuterol-sensitive delayed outward potassium currents in a cell model of spinal and bulbar muscular atrophy.Pflugers Archiv : European journal of physiology · 2021Article
- Article
- Motor Neuron Diseases and Neuroprotective Peptides: A Closer Look to Neurons.Frontiers in aging neuroscience · 2021Review
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
Abstract
Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by expansions of a polyglutamine (polyQ) tract in the androgen receptor (AR) gene. SBMA is associated with the progressive loss of lower motor neurons, together with muscle weakness and atrophy. PolyQ-AR is converted to a toxic species upon binding to its natural ligands, testosterone, and dihydrotestosterone (DHT). Our previous patch-clamp studies on a motor neuron-derived cell model of SBMA showed alterations in voltage-gated ion currents. Here, we identified and characterized chloride currents most likely belonging to the chloride channel-2 (ClC-2) subfamily, which showed significantly increased amplitudes in the SBMA cells. The treatment with the pituitary adenylyl cyclase-activating polypeptide (PACAP), a neuropeptide with a proven protective effect in a mouse model of SBMA, recovered chloride channel current alterations in SBMA cells. These observations suggest that the CIC-2 currents are affected in SBMA, an alteration that may contribute and potentially determine the pathophysiology of the disease.
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Registered trials
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