Dr. Kopp leads a translational research group within the Kidney Disease Section, Kidney Diseases Branch, studying focal segmental glomerulosclerosis (FSGS) and related podocyte diseases.
- Chromosome 22 harbors a major risk locus for kidney disease in African Americans, including FSGS, HIV-associated nephropathy, and arterionephroclerosis (hypertension-attributed kidney disease). APOL1 coding variants, which protect against trypanosomal infection, are strongly associated with kidney disease (odds ratios 7-29). The mechanism of glomerular injury is unknown.
- The HIV-1 protein Vpr, expressed in the glomerular podocytes, is sufficient to reproduce the chief features of HIV-associated collapsing glomerulopathy in transgenic mice.
Current research efforts
- determining the mechanisms by which Apol1 variants damage the glomerulus
- examining whether cardiotrophin-like cytokine 1 is a permeability factor that contributes to recurrent FSGS following kidney transplant
- an open label phase II trial examining the efficacy of rituximab combined with cyclosporine (for 48 weeks) for treatment of refractory podocyte disease
- participating in the ORD-funded NEPTUNE study of nephrotic diseases
Information for patients
- We are actively recruiting individuals with focal segmental glomerulosclerosis and those with undiagnosed nephrotic syndrome or proteinuria.
- All NIH trials are listed at clinicaltrials.gov.
- Information about glomerular diseases is available on the Clomerular Disease Primer page.
Reagents available to the research community
- Podocin promoter/rTTA (reverse tetracycline transactivator)—also available from JAX and as herozygotes or homozygotes
- TRE (tet responsive element)/Vpr
- Alb/TGF-beta mice (request permission from Dr. Snorri Thorgeirsson, NCI)
- rabbit polyclonal antibody to Vpr1-50 peptide—also available from AIDS Research and Reference Reagent Program
- rabbit antiserum to human podocin (cross-reactive with mouse podocin)
- rabbit antiserum to human nephrin (no cross-reactivity with mouse nephrin)
- goat antimouse mesangial cell serum, for induction of glomerulonephritis in mice
Podocyte cell lines
- mouse podocytes, immortalized with thermosensitive SV40 T Ag and bearing podocin/rtTA, for expression of genes of interest in cultured mouse podocytes
- same, plus TRE silencer to reduce background expression
- human urine derived podocyte-like epithelial cells (HUPECs), immortalized with hTERT and thermosensitive SV40 T Ag
Please contact us for further details. NIDDK MTAs are available through Technology Advancement and Transfer.