Intractable Rare Dis Res. 2025;14(2):148-150. (DOI: 10.5582/irdr.2025.01019)

A case of mitochondrial diabetes mellitus with successful therapeutic response following the initiation of imeglimin

Ishimura A, Suzuki S

Mitochondria are present in cells throughout the body and play a crucial role in energy production. They contain their own DNA, and mutations in this DNA can lead to a reduction in pancreatic beta cells and decreased insulin secretion, contributing to the development of diabetes. Insulin therapy has been considered a rational treatment, as the primary issue is impaired insulin secretion, but it primarily serves as a coping mechanism. Recently, however, imeglimin ‒ a drug believed to influence various mitochondria-mediated processes ‒ has been introduced and is expected to offer therapeutic benefits for mitochondrial diabetes. Here, we report a case of successful glycemic control following the addition of imeglimin in a patient with mitochondrial diabetes mellitus. After starting imeglimin, the patient's blood glucose levels stabilized, and he continues treatment. While the molecular target of imeglimin remains unknown, it is possible that the drug may offer significant benefits for patients with mitochondrial diabetes mellitus.

Full Text: