Target intelligence / Profile preview

Claudin-19 (CLDN19)

Target
CLDN19
Molecular classification
Tight junction protein, Transmembrane protein, Structural molecule, Barrier-forming protein
01

Overview

Claudin-19 is a tetraspan transmembrane protein and a critical structural component of tight junctions. It regulates paracellular permeability mainly for cations, contributing to the maintenance of epithelial barrier integrity in the kidney, retina, and peripheral nerves. In the kidney, claudin-19 coassembles with claudin-16, forming cation-selective channels, essential for the reabsorption of magnesium and calcium in the thick ascending limb of Henle’s loop. Claudin-19 also plays a role in regulating barrier properties in the retinal pigment epithelium, necessary for normal retinal tissue differentiation and vision. Genetic mutations in CLDN19 cause a rare inherited syndrome known as familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), often associated with severe ocular abnormalities such as visual impairment and nystagmus. The protein is not a current drug target, but is a key diagnostic biomarker for certain renal and ocular diseases.

Other names
CLDN19Claudin-19HOMG5claudin-19
02

Mechanism of action

Not applicable; no drugs are reported to directly target this molecule

03

Biological functions

Maintenance of epithelial barrierRegulation of paracellular ion transport (magnesium, calcium)Contribution to cell-cell adhesionMaintenance of ion gradientsElectrical sealing in the peripheral nervous systemRetinal tissue differentiation
04

Disease associations

Renal hypomagnesemiaOcular disease/visual impairmentNephrocalcinosisPeripheral neuropathy (in animal models)
05

Safety considerations

Genetic mutations result in severe renal and ocular abnormalities, including chronic renal failure and near blindnessNo safety concerns reported for therapeutic targeting, but disruption carries risk of impaired barrier function and electrolyte imbalances
06

Biomarkers

Mutations in CLDN19 are diagnostic biomarkers for familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), often presenting with ocular defects

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