Diet Patterns-A Neglected Aspect of Hemodialysis Care.
Published in Journal of the American Society of Nephrology : JASN
The Role of Intercalated Cell Nedd4-2 in BP Regulation, Ion Transport, and Transporter Expression.
...Published in Journal of the American Society of Nephrology : JASN
BackgroundNedd4-2 is an E3 ubiquitin-protein ligase that associates with transport proteins, causing their ubiquitylation, and then internalization and degradation. Previous research has suggested a correlation between Nedd4-2 and BP. In this study, we explored the effect of intercalated cell (IC) Nedd4-2 gene ablation on IC transporter abundance a...
The Underestimated Burden of Monogenic Diseases in Adult-Onset ESRD.
Published in Journal of the American Society of Nephrology : JASN
Potentiating Tissue-Resident Type 2 Innate Lymphoid Cells by IL-33 to Prevent Renal Ischemia-Reperfusion Injury.
...Published in Journal of the American Society of Nephrology : JASN
The IL-33-type 2 innate lymphoid cell (ILC2) axis has an important role in tissue homeostasis, inflammation, and wound healing. However, the relative importance of this innate immune pathway for immunotherapy against inflammation and tissue damage remains unclear. Here, we show that treatment with recombinant mouse IL-33 prevented renal structural ...
ApoL1 Overexpression Drives Variant-Independent Cytotoxicity.
...Published in Journal of the American Society of Nephrology : JASN
Coding variants in the APOL1 gene are associated with kidney diseases in African ancestral populations; yet, the underlying biologic mechanisms remain uncertain. Variant-dependent autophagic and cytotoxic cell death have been proposed as pathogenic pathways mediating kidney injury. To examine this possibility, we conditionally expressed APOL1-G0 (r...
Maternal and Fetal Outcomes of Pregnancies in Women with Atypical Hemolytic Uremic Syndrome.
...Published in Journal of the American Society of Nephrology : JASN
Atypical HUS (aHUS) is a disorder most commonly caused by inherited defects of the alternative pathway of complement, or the proteins that regulate this pathway, and life-threatening episodes of aHUS can be provoked by pregnancy. We retrospectively and prospectively investigated 27 maternal and fetal pregnancy outcomes in 14 women with aHUS from th...
Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach.
Published in Journal of the American Society of Nephrology : JASN
FSGS describes a renal histologic lesion with diverse causes and pathogenicities that are linked by podocyte injury and depletion. Subclasses of FSGS include primary, genetic, and secondary forms, the latter comprising maladaptive, viral, and drug-induced FSGS. Despite sharing certain clinical and histologic features, these subclasses differ notice...
Control of Humoral Response in Renal Transplantation by Belatacept Depends on a Direct Effect on B Cells and Impaired T ...
...Published in Journal of the American Society of Nephrology : JASN
Generation of de novo donor-specific antibodies (dnDSAs) after renal transplant is recognized as the leading cause of late transplant failure. Hence, the optimal immunosuppressive strategies to limit dnDSA development need to be defined. Recent clinical trials using the novel costimulatory blockade agent CTLA4-Ig (Belatacept) have shown that kidney...
Targeting B Cells and Plasma Cells in Glomerular Diseases: Translational Perspectives.
Published in Journal of the American Society of Nephrology : JASN
The unique contributions of memory B cells and plasma cells in kidney diseases remain unclear. In this review, we evaluate the clinical experience with treatments directed at B cells, such as rituximab, and at plasma cells, such as proteasome inhibition, to shed light on the role of these two B lineage compartments in glomerular diseases. Specifica...
Pathogenicity of a Human Laminin β2 Mutation Revealed in Models of Alport Syndrome.
Published in Journal of the American Society of Nephrology : JASN
Pierson syndrome is a congenital nephrotic syndrome with eye and neurologic defects caused by mutations in laminin β2 (LAMB2), a major component of the glomerular basement membrane (GBM). Pathogenic missense mutations in human LAMB2 cluster in or near the laminin amino-terminal (LN) domain, a domain required for extracellular polymerization of lami...
