Renal fibrosis is found in genetic kidney diseases. In some of them, fibros
is may be due to mechanisms not dependent on the gene defect. In other case
s, fibrosis depends more specifically on the gene defect: e.g. in juvenile
nephronophthisis where interstitial fibrosis predominates, or in Alport's s
yndrome where glomerulosclerosis is triggered probably by unbalanced distri
bution of a chains of type IV collagen in glomerular basement membrane. Stu
dy of genes predisposing to renal fibrosis and progression is more complex.
Genes regulating the renin-angiotensin system have been considered. The DD
polymorphism of the converting enzyme gene may be associated with a more r
apid progression rate of renal failure ia glomerular and non-glomerular dis
eases. This remains, however, to be confirmed in more appropriate studies.