Plekhh2, a novel podocyte protein downregulated in human focal segmental glomerulosclerosis, is involved in matrix adhesion and actin dynamics.

Pleckstrin homology domain-containing, family H (with MyTH4 domain), member 2 (Plekhh2) is a 1491-residue intracellular protein highly enriched in renal glomerular podocytes for which no function has been ascribed. Analysis of renal biopsies from patients with focal segmental glomerulosclerosis revealed a significant reduction in total podocyte Plekhh2 expression compared to controls. Sequence analysis indicated a putative α-helical coiled-coil segment as the only recognizable domain within the N-terminal half of the polypeptide, while the C-terminal half contains two PH, a MyTH4, and a FERM domain.

Pdlim2 is a novel actin-regulating protein of podocyte foot processes.

The slit diaphragm and the apical and basal membrane domains of podocytes are connected to each other by an actin-based cytoskeleton critical to the maintenance of the glomerular filtration barrier. In an effort to discover novel regulatory proteins of the podocyte foot process, we identified and characterized pdlim2, a member of the actin-associated LIM protein subfamily of cytosolic proteins typified by an N-terminal PDZ domain and a C-terminal LIM domain. In the kidney, the pdlim2 protein is highly specific for the glomerulus and podocyte foot processes as shown by RT-PCR, western blotting, immunofluorescence, and immunoelectron microscopy.

Permeability, ultrastructural changes, and distribution of novel proteins in the glomerular barrier in early puromycin aminonucleoside nephrosis.

Background/aims: It is still unclear what happens in the glomerulus when proteinuria starts. Using puromycin aminonucleoside nephrosis (PAN) rats, we studied early ultrastructural and permeability changes in relation to the expression of the podocyte-associated molecules nephrin, α-actinin, dendrin, and plekhh2, the last two of which were only recently discovered in podocytes.

Methods: Using immune stainings, semiquantitative measurement was performed under the electron microscope.

Dendrin expression in glomerulogenesis and in human minimal change nephrotic syndrome.

Background: Dendrin is an 81-kD cytosolic protein hitherto described in the brain, where it is associated with the actin cytoskeleton. Recently, we found dendrin in foot processes of mouse glomerular podocytes. Here we describe its expression both during mouse glomerulogenesis and in the normal and diseased human kidney for the first time.

Altered ultrastructural distribution of nephrin in minimal change nephrotic syndrome.

Background: Nephrin is a cell-adhesion protein that is defective in congenital nephrotic syndrome of the Finnish type (CNF). Nephrin is synthesized by the podocytes and is localized to the slit membrane between individual foot processes of the podocytes. Although nephrin is apparently pivotal in the development of CNF, the role of nephrin in other causes of nephrotic syndrome is not fully understood.

No evidence of accelerated loss of kidney function in living kidney donors: results from a cross-sectional follow-up.

Background: There is a lack of kidneys available for kidney transplantation, and living donors are increasingly used. It is important to examine the possible long-term adverse affect on the renal function and blood pressure of the donors.

Methods: We have made a comprehensive follow-up of all living kidney donors at our center from 1964 to 1995.

Kidney donors don't regret: follow-up of 370 donors in Stockholm since 1964.

Background: The aim of the study was to present the views of our kidney donors since 1964, at the time of donation, as well as later on--and to assess their current subjective health.

Methods: A total of 451 living-donor nephrectomies were performed on Swedish residents in Stockholm from April 1964 until the end of 1995. A questionnaire with 11 questions about the donation and a standardized health form (SF-36) were sent to all donors alive in 1997 (n=403).