Human genetic studies over the past few decades have confirmed that FSGS is primarily a podocytopathy
and that more than 20 mutated genes are implicated in the pathogenesis of NS/FSGS. NGS is rapidly transforming the genetic testing of FSGS. WES will likely be available as a clinical diagnostic at a much lower cost, which will enable the convenient analysis of multiple FSGS-related podocyte genes and the exploration of genetic factors in the pathogenesis of FSGS.
(14) The heavy proteinuria possibly relates to podocytopathy
, as the histological changes are not sufficiently severe to explain this degree of proteinuria.
The first episode occurred with biopsy findings consistent with lupus nephritis (LN) class V (membranous) without clinical evidences of systemic lupus erythematosus (SLE), and the second episode occurred with pathologic findings of a nonimmune complex podocytopathy
on the repeat kidney biopsy, subsequently with positive serologic evidences of SLE.
Devaraj, "Global toll-like receptor 4 knockout results in decreased renal inflammation, fibrosis and podocytopathy
," Journal of Diabetes and its Complications, vol.
Johnson, "Minimal change disease: a CD80 podocytopathy
?," Seminars in Nephrology, vol.
MCD is, therefore, also classified as a type of primary podocytopathy
Interestingly, accumulating data revealed that podocytopathy
plays a fundamental role in kidney diseases associated with metabolic disorders such as diabetic kidney disease, salt-sensitive hypertension, and obesity-related glomerulopathy [27-31].
Wolf, "Pathogenesis of the podocytopathy
and proteinuria in diabetic glomerulopathy," Current Diabetes Reviews, vol.
Mundel, "Role of angiotensin II in the development of nephropathy and podocytopathy
of diabetes," Current Diabetes Reviews, vol.
However, in previous reports, some patients with lupus nephritis presented with "scanty immune deposits," that is, nonclassical glomerulonephritis, which might contribute to thrombotic microangiopathy (TMA) , ANCA-associated crescentic glomerulonephritis  podocytopathy
, and so forth.
It is a podocytopathy
with distinct clinicopathologic features from focal segmental glomerulosclerosis as the lesions are characterized by formation of pseudocrescents and by collapse of capillary loops rather than extracellular matrix accumulation and glomerulosclerosis being a late manifestation.
Figure 1 illustrates the basic cellular organization of the podocyte foot process, with specific emphasis upon the localization and the protein-protein associations amongst the soon to be discussed biomarkers of podocytopathy