Patients with a rare, genetic form of diabetes often are misdiagnosed as having Type 2 diabetes and, as a result, may be at risk for dangerous complications because they may receive improper treatment.

That’s the upshot of a new study out of Washington University School of Medicine in St. Louis that suggests that diabetics with the genetic form of the disease —called maturity-onset diabetes of the young (MODY1) — are very different from those with Type 2 diabetes.

The study, published in in The Journal of Biological Chemistry, suggests treating MODY1 patients with therapies designed for Type 2 diabetes is potentially harmful an can lead to the destruction of insulin-secreting beta cells that regulate blood sugar.

"People diagnosed with Type 2 diabetes are treated with oral medications that make insulin-secreting beta cells very active," said Benjamin D. Moore, a former postdoctoral fellow at Washington University who is now at Massachusetts General Hospital.

"But the MODY1 pathway we've uncovered shows that stimulating those cells with those drugs can lead to beta cell death. That means these patients can become dependent on insulin injections much sooner."

Moore said it's common for patients with MODY1, who make up 3 percent to 5 percent of all patients with diabetes, to transition from oral medications to insulin injections within 10 years of diagnosis as a way to keep their blood sugar in check.

But the new research suggests those with MODY1 might benefit from different therapies that target a specific pathway that appears to be essential to the function of insulin-secreting cells.

"It's important to diagnose patients as accurately as possible and to attempt to target the correct pathway," Moore said.

The study was funded, in part, by the National Institute of Diabetes and Digestive and Kidney Diseases of the National Institutes of Health.