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Table of Contents
Definition / general | Clinical features | Uses by pathologists | Microscopic (histologic) images | Positive staining - normal | Electron microscopy imagesCite this page: Nephrin (NPHS1). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/stainsnephrin.html. Accessed April 24th, 2018.
Definition / general
- Product of NPHS1 gene; discovered when NPHS1 mutations were found in patients with congenital nephrotic syndrome of the Finnish type (Mol Cell 1998;1:575)
- 180 kDa transmembrane protein localized on lateral surface of podocyte foot process (Patholog Res Int 2013;2013:782395)
- Podocyte marker and key component of slit-diaphragm (Front Endocrinol (Lausanne) 2014;5:148)
Clinical features
- Claudin1 and nephrin label cellular crescents in diabetic glomerulosclerosis (Hum Pathol 2014;45:628)
- Nephrin phosphorylation helps maintain normal structure and function in podocytes (Mol Biol Rep 2014;41:3591), which may be mechanism of action of dexamethasone in glomerulonephritis
- Mutations that affect nephrin expression / function often affect podocytes and cause chronic nephropathies (Int J Nephrol 2013;2013:4574909, Physiol Rep 2013;1:e00086, Pak J Biol Sci 2013;16:882, Nephron Clin Pract 2012;120:c139)
- Urinary nephrin may predict preeclampsia and small-for-gestational age infants (Obstet Gynecol Sci 2013;56:22, Eur J Obstet Gynecol Reprod Biol 2013;166:139)
Uses by pathologists
- No specific uses at this time
Microscopic (histologic) images
Diabetic nephropathy
Nature images:
Progression of renal damage, NPHS1 kidneys
Tubules in NPHS1 patients
Inflammatory cells, tubulointerstitium
Positive staining - normal
- Podocytes, also CNS and β cells of islets of Langerhans (J Biol Chem 2012;287:28932)
Electron microscopy images
Nature image:
Kidney
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