Resources for PKD research
A. High-throughput barcoded mutation analysis of all ~1,500 exons of ~90 human renal cystic genes
B. High-throughput barcoded mutation analysis of all ~1,500 exons of ~90 mouse renal cystic genes
C. Whole exome sequencing (WES) of smaller cohorts of human patients
D. Sequencing of gene of interest (candidate PKD modifier or ciliopathy gene) in an existing cohort of PKD-ciliopathy patients
A. Full-length DNA constructs for the expression of normal and mutant PKD proteins (PKD1, PKD2, and PKHD1)
B. Stable cell lines of the full-length PKD1, PKD2, and PKHD1 constructs
C. Monoclonal and polyclonal antibodies against different regions of PC1, PC2 and FPC proteins (if there is a specific domain/type you are intested, please indicate)
D. Kidney tissues, tissue sections, and cystic fluids from normal and ADPKD patients
E. hTERT immortalized renal tubular epithelial cells from ADPKD patients
F. Pkd1 and Pkd2 floxed or germline mutant mice with or without inducible Cre transgene
G. Tissue sections and kidney tissues from wild type and Pkd1 and Pkd2 knockout mice induced at different stages.
H. Primary culture of renal epithelial cells from Pkd1f/f and Pkd2f/f mouse model.
I. Inducible immortalized cell lines from Pkd1f/f and Pkd2f/f mouse model using mouse telomerase reverse transcriptase (mTert).
J. Provide consultations, protocols, and hand-by-hand teaching to investigators who wish to learn kidney cell culture and three-dimensional culture (cystogenesis and tubulogensis assay) using renal tubular epithelial cells.
A. iPS Cell Generation
Distribution of existing iPS cells from patients with ADPKD/ARPKD/ciliopathies
Derivation of new iPS cells from PKD/ciliopathy patient cells (e.g. fibroblasts or kidney epithelial cells)
In vitro characterization of iPS cells to demonstrate pluripotency
Teratoma formation from iPS cells in immunodeficient mice
B. Genome Engineering
Generation of CRISPR constructs for PKD/ciliopathy disease genes
Derivation of mutant iPS cell lines using CRISPRs
C. Directed Differentiation
Differentiation of iPS cells into kidney progenitor cellsDifferentiation of iPS cells into liver progenitor cells
Biological samples (RNA and protein) from iPS cells or derived tissues
Complex organoid formation from iPS cells in vitro
Implantation of iPS-derived somatic cells into liver and kidney of immunodeficient mice
Derivation of mutant somatic cell lines using CRISPRs
Differentiation of iPS cells into endothelial and myocardial progenitor cells
Banking and distribution of iPS cells for other investigators