Nephrin is a
protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, respo ...
necessary for the proper functioning of the renal filtration barrier. The renal filtration barrier consists of fenestrated endothelial cells, the glomerular basement membrane, and the
podocytes
Podocytes are cells in Bowman's capsule in the kidneys that wrap around capillaries of the glomerulus. Podocytes make up the epithelial lining of Bowman's capsule, the third layer through which filtration of blood takes place. Bowman's capsule f ...
of epithelial cells. Nephrin is a transmembrane protein that is a structural component of the
slit diaphragm.
They are present on the tips of the podocytes as an intricate mesh and convey strong negative charges which repel protein from crossing into the
Bowman's space.
A defect in the gene for nephrin, NPHS1, is associated with
congenital nephrotic syndrome
Congenital nephrotic syndrome is a rare kidney disease which manifests in infants during the first 3 months of life, and is characterized by high levels of protein in the urine (proteinuria), low levels of protein in the blood, and swelling. This ...
of
the Finnish type and causes massive amounts of protein to be leaked into the urine, or
proteinuria
Proteinuria is the presence of excess proteins in the urine. In healthy persons, urine contains very little protein; an excess is suggestive of illness. Excess protein in the urine often causes the urine to become foamy (although this symptom ma ...
. Nephrin is also required for cardiovascular development.
Interactions
Nephrin has been shown to
interact
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with:
*
CASK
A barrel or cask is a hollow cylindrical container with a bulging center, longer than it is wide. They are traditionally made of wooden staves and bound by wooden or metal hoops. The word vat is often used for large containers for liquids, u ...
,
[
* ]CD2AP
CD2-associated protein is a protein that in humans is encoded by the ''CD2AP'' gene.
Function
This gene encodes a scaffolding molecule that regulates the actin cytoskeleton. The protein directly interacts with filamentous actin and a variety o ...
,[ and
* ]CTNND1
p120, and called catenin delta-1 is a protein that in humans is encoded by the CTNND1 gene.
Function
This gene encodes a member of the Armadillo protein family, which function in adhesion between cells and signal transduction. Multiple transla ...
,[
* ]FYN
Funen ( da, Fyn, ), with an area of , is the third-largest island of Denmark, after Zealand and Vendsyssel-Thy. It is the 165th-largest island in the world. It is located in the central part of the country and has a population of 469,947 as ...
,KIRREL
Kin of IRRE-like protein 1, also known as NEPH1, is a protein that in humans is encoded by the ''KIRREL'' gene.
Function
NEPH1 is a member of the NEPH protein family, which includes NEPH2 ( KIRREL3, MIMbr>607761 and NEPH3 ( KIRREL2, MIMbr>6077 ...
,NPHS2
Podocin is a protein that in humans is encoded by the ''NPHS2'' gene.
Interactions
NPHS2 has been shown to interact with Nephrin and CD2AP.
See also
* Focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis (FSGS) is a histo ...
.
See also
* Podocyte
Podocytes are cells in Bowman's capsule in the kidneys that wrap around capillaries of the glomerulus. Podocytes make up the epithelial lining of Bowman's capsule, the third layer through which filtration of blood takes place. Bowman's capsule f ...
References
Further reading
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External links
* {{MeshName, nephrin
Proteins