FKFBP1A (FK506-binding protein 1A) is a peptidyl-prolyl cis-trans isomerase. It interacts with the ryanodine receptor (RyR1) and helps in maintaining intracellular Ca2+ homeostasis. It is also involved in protein folding, cell cycle and protein trafficking. In neurodegenerative diseases, FKFBP1A is dysregulated and might be associated with the pathogenesis. In human cell lines, it controls the localization and processing of amyloid precursor protein, and thus might be involved in the pathogenesis of Alzheimer′s disease. FKFBP1A is also involved in Huntington′s disease. It plays a role in the structural properties and proteotoxicity of mHTT (mutant huntingtin) aggregates.