FRRB Project 1745126 - A thorough understanding of the molecular players causing rare complement-mediated glomerular diseases toward a personalized therapy

Name and Surname of PI

Caterina Mele

Project Acronym


Project ID


Host Institution

Istituto di Ricerche Farmacologiche Mario Negri IRCCS - Milan

Pathology of Interest

C3 glomerulopathy / Immune-complex-mediated membranoproliferative glomerulonephritis

Research Area

Rare Diseases / Nephrology

Project Start Date

1 April 2021

Project End Date

31 March 2024


€ 526.000,00

Type of Project



Immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) are rare kidney disorders, which lack an effective treatment. The prognosis is unfavourable and most patients develop end-stage renal failure during late childhood and early adolescence. Genetic defects leading to an uncontrolled activation of the complement system are found in C3G as frequently as in IC-MPGN. However, the genetic causes are still unknown in approximately 80% of patients, and the functional consequences of the genetic abnormalities have been investigated only in a few cases. Using a multidisciplinary approach, we will study the molecular, biochemical and cellular mechanisms through which the genetic alterations cause IC-MPGN/C3G. The results of these studies will be instrumental in characterising the pathogenetic mechanism underlying the disease in each patient, and will contribute to the development of personalised therapies.