Effects of anserine on oxidative stress and on cell barrier integrity in methylmalonic aciduria

Area of Science:

• Nephrology
• Biochemistry
• Mitochondrial Biology

Background:

• Methylmalonyl-CoA mutase deficiency (mut0) causes kidney damage via metabolic and oxidative stress.
• Anserine possesses antioxidant properties and may protect kidney cells.
• This study investigates anserine's therapeutic potential in mut0 kidney disease.

Purpose of the Study:

• To evaluate anserine's effects on immortalized kidney tubular epithelial cells (iKTEC) from mut0 patients.
• To assess anserine's impact on cell viability, mitochondrial function, and epithelial integrity.
• To determine if anserine can mitigate oxidative stress in mut0 kidney cells.

Main Methods:

• Cultured iKTEC from mut0 patients and healthy controls.
• Assessed cell viability, antioxidant capacity, mitochondrial respiration, and ATP production.
• Measured zonula occludens-1 (ZO-1) expression, transepithelial resistance (TER), and dextran transport.

Main Results:

• mut0 iKTEC exhibited reduced viability, antioxidant capacity, oxygen consumption, and ATP production compared to controls.
• Anserine treatment restored antioxidant capacity and normalized ZO-1 expression in mut0 iKTEC.
• Anserine did not significantly affect cell viability, mitochondrial respiration, ATP production, TER, or dextran transport.

Conclusions:

• Anserine demonstrates potential in restoring antioxidant capacity in mut0 kidney cells.
• Anserine may serve as a therapeutic agent to combat reactive oxygen species (ROS) in mut0 disease.
• Further investigation is required to understand anserine's effects on mitochondrial function and epithelial integrity in this context.