Journal
FRONTIERS OF MEDICINE
Volume 11, Issue 3, Pages 333-339Publisher
SPRINGER
DOI: 10.1007/s11684-017-0564-1
Keywords
nephrotic syndrome; focal segmental glomerulosclerosis; genetic
Categories
Funding
- National Project for the Construction of Clinical Key Specialty
- Project of Special Fund for Health-Scientific Research [201002010]
- National Key Technology RD Program [2011BAI10B00]
- National Basic Research Program of China [2012CB517700, 2012CB517604]
- National Natural Science Foundation of China [81270782, 30771000]
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Nephrotic syndrome (NS) is one of the most common glomerular diseases with signs of nephrosis, heavy proteinuria, hypoalbuminemia, and edema. Dysfunction of glomerular filtration barrier causes protein loss through the kidneys. Focal segmental glomerulosclerosis (FSGS) accounts for nearly 20% of NS among children and adults. Adult-onset FSGS/NS is often associated with low response to steroid treatment and immunosuppressive medication and poor renal survival. Several genes involved in NS and FSGS have been identified by linkage analysis and next-generation sequencing. Most of these genes encode proteins and are highly expressed in glomerular podocytes, which play crucial roles in slit-diaphragm signaling, regulation of actin cytoskeleton dynamics and maintenance of podocyte integrity, and cell-matrix interactions. In this review, we focus on the recently identified genes in the adult-onset NS and FSGS and discuss clinical significance of screening of these genes.
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