While it is not fully characterized as such, yet,<unk>  seems to be an uncharacterized form of ciliopathy. A ciliopathy is a disease that affects the cilia (sensing cells within the body). The link to ciliar problems as a cause for Nephronopthisis and similar Kidney diseases is relatively new. Watnick and Germino note that NPHP1 and NPHP4 encode for the proteins nephrocystin and nephrocystin-4 (nephroretinin). These have been shown to interact in a series of cell-cell and cell-matrix signaling proteins. NPHP2 has been also shown to have possible links to the function of the primary renal cilium and to control of the cell cycle (Otto). Otto further found that nephrocystin, inversin (INVS) and nephroretinin colocalize in the primary cilia of cultured renal epithelia cells. One interesting connection is that primary cilia in renal cells may perform a sensing function which maintains the renal tubules. The loss of cystoproteins may lead to dysregulated growth. The link to a ciliar dysfunction in the Retinal degenerative diseases also comes from Otto. Like the described Kidney diseases, Retinitis Pigmentosa is a disease where the ciliar cells (Rods and Cones) fail to thrive. In a study of patients with a disease similar to<unk>  (renal-retinal Senior-Loken) the authors state "We show that nephrocystin-5, RPGR and calmodulin can be coimmniprecipitated from retinal extracts, and that these proteins localize to connecting cilia of photoreceptors and to primary cilia of renal epithelial cells. Our studies emphasize the central role of ciliary dysfunction in the pathogenesis of renal-retinal Senior Loken Syndrome." In other words, there is a common link between certain kidney diseases and some forms of RP and it is through the something related to a problem in the cilia cells. This may be a problem in the cells themselves, or with something that keeps them alive and healthy.
Conorenal syndrome
Papillorenal syndrome
Hepatorenal syndrome
Cardiorenal syndrome
Acute uric acid nephropathy
Dialysis disequilibrium syndrome
Contrast-induced nephropathy
Analgesic nephropathy