Updated publication reference for PubMed record(s): 31461451. Hyperinsulinemia affects 72% of Fanconi anemia (FA) patients and an additional 25% experience lowered glucose tolerance or frank diabetes. The underlying molecular mechanisms contributing to the dysfunction of FA pancreas β cells is unknown. Therefore, we sought to identify previously unexplored roles of FA proteins in the glucose responsive human pancreas β cell line EndoC-βH3 using a mass spectrometry-based protein interaction analysis of endogenous FANCA. This study reveals glucose stimulation-dependent changes in the FANCA interaction network indicative of the DNA damage response (DDR) through interactions with other FA proteins. We identify protein interactions with FANCA related to β cell insulin secretion.