Rare Form of Diabetes Requires Different Treatment Strategy


Administering type 2 diabetes drugs to patients with maturity-onset diabetes of the young can cause harm.

Patients with a rare form of diabetes can often be misdiagnosed with type 2 diabetes because of similarities between the conditions. However, new research has shown that therapies meant for type 2 patients can actually cause them harm, and the guidelines should be changed, according to a recent study.

The genetic form is called maturity-onset diabetes of the young (MODY1). If patients are treated with medication meant for patients suffering from type 2 diabetes, it could lead to the destruction of insulin-secreting beta cells responsible for regulating blood sugar.

“People diagnosed with type 2 diabetes are treated with oral medications that make insulin-secreting beta cells very active,” said first study author Benjamin D. Moore, PhD. “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.”

MODY1 accounts for 3% to 5% of all patients with diabetes and these individuals will typically transition from oral medications to insulin injections within 10 years of diagnosis.

However, researchers from the Washington University School of Medicine found that oral medications that increase beta cells only increase cellular stress levels, resulting in the death of those cells and causing patients to switch up the insulin.

The study, published in The Journal of Biological Chemistry, reported patients with MODY1 would see greater benefit from therapies that target specific pathways necessary for the function of insulin-secreting cells.

Additionally, researchers saw a relationship between a pair of proteins that regulate cells and secrete enzymes and insulin in the intestines, kidney, liver, and stomach. Out of the pair, 1 of the proteins is developed from the gene that is altered in MODY1 diabetes patients.

“Nature doesn't re-invent the wheel,” said researcher Jason C. Mills, MD, PhD. “What these different cells secrete is very different, but the machinery is very similar. As with auto plants, although a BMW is very different from a Volkswagen, the factories where those cars are built are not really that different. It appears the same thing may be true for a number of these cells that secrete key enzymes in the gastrointestinal tract.”

Currently, researchers are continuing their studies to determine if the same pathways are active in different types of secretory cells. Additionally, researchers stress that physicians need to evaluate whether patients have MODY1 diabetes or not before starting treatment.

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

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