Huntingtin-Associated Protein 1A Regulates Store-Operated Calcium Entry in Medium Spiny Neurons From Transgenic YAC128 Mice, a Model of Huntington's Disease.

ABSTRACT: Huntington's disease (HD) is a hereditary neurodegenerative disease that is caused by polyglutamine expansion within the huntingtin (HTT) gene. One of the cellular activities that is dysregulated in HD is store-operated calcium entry (SOCE), a process by which Ca²⁺ release from the endoplasmic reticulum (ER) induces Ca²⁺ influx from the extracellular space. HTT-associated protein-1 (HAP1) is a binding partner of HTT. The aim of the present study was to examine the role of HAP1A protein in regulating SOCE in YAC128 mice, a transgenic model of HD. After Ca²⁺ depletion from the ER by the activation of inositol-(1,4,5)triphosphate receptor type 1 (IP₃R1), we detected an increase in the activity of SOC channels when HAP1 protein isoform HAP1A was over

SUBMITTER: Czeredys M 

PROVIDER: S-EPMC6231533 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature