UMOD gene
The UMOD gene provides instructions for making uromodulin, a protein essential for kidney function and the regulation of minerals in urine.
Normal Function
The UMOD gene provides instructions for making a protein called uromodulin, which is produced exclusively in the loop of Henle in the kidney. This area of the kidney is responsible for reabsorbing nutrients and minerals to prevent their loss in urine.
Uromodulin functions include:
- Assisting in the transport of sodium and potassium to help control water levels in urine.
- Maintaining and protecting the lining of the loop of Henle.
- Potentially protecting the body against urinary tract infections and kidney stones.
Health Conditions Related to Genetic Changes
Autosomal dominant tubulointerstitial kidney disease-UMOD
Variants in the UMOD gene cause autosomal dominant tubulointerstitial kidney disease-UMOD (ADTKD-UMOD), an inherited condition leading to progressive kidney disease.
The disease mechanism involves:
- Structural changes: Gene variants alter the structure of uromodulin, preventing it from reaching its required location in the loop of Henle.
- Transport interference: A lack of functional uromodulin disrupts the transport of sodium and potassium.
- Cell damage: The buildup of abnormal uromodulin may trigger the self-destruction (apoptosis) of kidney cells, ultimately leading to kidney failure.
