Increased glycosphingolipid excretion associated with proteinuria.

Townsend, Raymond R.; Orth, Roger; Clawson, C M; Li, S C; Li, Y T

doi:10.1172/jci109095

Cited by 6 publications

(2 citation statements)

References 19 publications

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“…Glycosphingolipiduria occurs in the presence of proteinuria of various aetiologies 61 but whether it contributes to disease progression is yet to be established. Some glycosphingolipids act as regulators of outside-in signalling.…”

Section: Sphingolipids and Gangliosidesmentioning

confidence: 99%

Lipid biology of the podocyte—new perspectives offer new opportunities

2014

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In the past 15 years, major advances have been made in understanding the role of lipids in podocyte biology. First, susceptibility to focal segmental glomerulosclerosis (FSGS) and glomerular disease is associated with an APOL1 sequence variant, is expressed in podocytes and encodes apolipoprotein L1, an important component of HDL. Second, acid sphingomyelinase-like phosphodiesterase 3b encoded by SMPDL3b has a role in the conversion of sphingomyelin to ceramide and its levels are reduced in renal biopsy samples from patients with recurrent FSGS. Furthermore, decreased SMPDL3b expression is associated with increased susceptibility of podocytes to injury after exposure to sera from these patients. Third, in many individuals with membranous nephropathy, autoantibodies against the phospholipase A2 (PLA2) receptor, which is expressed in podocytes, have been identified. Whether these autoantibodies affect the activity of PLA2, which liberates arachidonic acid from glycerophospholipids and modulates podocyte function, is unknown. Fourth, clinical and experimental evidence support a role for ATP-binding cassette sub-family A member 1-dependent cholesterol efflux, free fatty acids and glycerophospolipids in the pathogenesis of diabetic kidney disease. An improved understanding of lipid biology in podocytes might provide insights to develop therapeutic targets for primary and secondary glomerulopathies.

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Section: Sphingolipids and Gangliosidesmentioning

confidence: 99%

Lipid biology of the podocyte—new perspectives offer new opportunities

2014

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“…Data from previous studies indicate that Gb3 may be increased in plasma and/or urine in other diseases as well [24,25]. In as early as 1978, reports on urinary glycosphingolipids in the urine of patients without sphingolipidoses were published [31]. The specificity of the diagnostic criteria that were part of this study and that (in)directly assess Gb3 in urine ('immunohistochemical staining of Gb3 in urine', 'Maltese cross sign in urine' and 'high urinary Gb3 (in range of classical males)') was therefore debated by the panel.…”

Section: Discussionmentioning

confidence: 99%