New insights into the pathogenesis and the therapy of recurrent focal glomerulosclerosis.

Abstract

Recurrent focal glomerulosclerosis (FSGS) in renal allografts has remained a frustrating and enigmatic disease. Recent studies on gene mutations encoding podocin and other components of the slit-diaphragm in patients with native kidney nephrotic syndrome have underscored the heterogenecity of the idiopathic form of FSGS. While familial FSGS rarely recurs following transplantation, the sporadic variety of FSGS is associated with a 30% recurrence rate. The patients with the sporadic variety of FSGS who have homozygous or complex heterozygous podocin mutations have a low recurrence rate. In the other patients with sporadic FSGS, a more complex and likely multifactorial etiology accounts for the recurrence of FSGS. The role of CD80 expression on podocytes is intriguing but requires confirmation in kidney biopsies of patients with recurrent FSGS. Recent findings on podocin genomics, the permeability factor and CD80 expression may ultimately lead to a better understanding of recurrent FSGS as well as a more effective approach to its prevention and treatment.

• Authors: Flavio Vincenti, Gian Marco Ghiggeri
• Journal: American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons (Am J Transplant) Vol. 5 Issue 6 Pg. 1179-85 (Jun 2005) ISSN: 1600-6135 [Print] United States
• PMID: 15888021 (Publication Type: Journal Article, Review)

Chemical References

• Intracellular Signaling Peptides and Proteins
• Membrane Proteins
• NPHS2 protein

Topics

• Genetic Predisposition to Disease
• Glomerulosclerosis, Focal Segmental (etiology, therapy)
• Humans
• Intracellular Signaling Peptides and Proteins
• Kidney Transplantation
• Membrane Proteins (genetics)
• Mutation (genetics)
• Recurrence