A Novel Role for GATA3 in Mesangial Cells in Glomerular Development and Injury

Grigorieva, I. V.; Oszwald, André; Grigorieva, Elena; Schachner, Helga; Neudert, Barbara; Ostendorf, Tammo; Floege, Jürgen; Lindenmeyer, Maja T.; Cohen, Clemens D.; Panzer, Ulf; Aigner, Christof; Schmidt, Alice; Grosveld, Frank; Thakker, Rajesh; Rees, Andrew J.; Kain, Renate

doi:10.1681/asn.2018111143

Cited by 31 publications

(21 citation statements)

References 56 publications

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“…These findings are consistent with the observed hypocalcaemia that occurs in association with inappropriately normal or low-plasma PTH levels in patients who have the HDR syndrome due to GATA3 mutations (Grigorieva et al, 2010). More recently, heterozygous Gata3 mice were shown to have impaired mesangial cell progenitor migration and proliferation during kidney development resulting in fewer mesangial cells and glomerular abnormalities in adult mice (Grigorieva et al, 2019).…”

Section: Animal Modelssupporting

confidence: 86%

Hypoparathyroidism, deafness, and renal dysplasia syndrome: 20 Years after the identification of the firstGATA3mutations

Lemos

Thakker

2020

Human Mutation

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The hypoparathyroidism, deafness, and renal dysplasia (HDR) syndrome is an autosomal dominant disorder caused by heterozygous mutations of the GATA3 gene. In the last 20 years, since the identification of the genetic cause of the HDR syndrome, GATA3 mutations have been reported in 124 families (177 patients). The clinical aspects and molecular genetics of the HDR syndrome are reviewed here together with the reported mutations and phenotypes. Reported mutations consist of 40% frameshift deletions or insertions, 23% missense mutations, 14% nonsense mutations, 6% splice‐site mutations, 1% in‐frame deletions or insertions, 15% whole‐gene deletions, and 1% whole‐gene duplication. Missense mutations were found to cluster in the regions encoding the two GATA3 zinc‐finger domains. Patients showed great clinical variability and the penetrance of each HDR defect increased with age. The most frequently observed abnormality was deafness (93%), followed by hypoparathyroidism (87%) and renal defects (61%). The mean age of diagnosis of HDR was 15.3, 7.5, and 14.0 years, respectively. However, patients with whole‐gene deletions and protein‐truncating mutations were diagnosed earlier than patients with missense mutations.

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Section: Animal Modelssupporting

confidence: 86%

Hypoparathyroidism, deafness, and renal dysplasia syndrome: 20 Years after the identification of the firstGATA3mutations

Lemos

Thakker

2020

Human Mutation

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“…Black babies are twice as likely to die as white babies. 1 Black people choose hospice a third less often than white people and disenroll at higher rates. 2 Further, many a researcher has built an academic career pointing out these disparities, without ever suggesting real ways to overcome them.…”

Section: Nonementioning

confidence: 99%

“…Another significant finding from Grigorieva et al 1 is that GATA3 is a robust marker of healthy and diseased mesangial nuclei in both mouse and human glomeruli. This nuclear localization allows for facile quantification of mesangial cell number, avoiding issues of cell segmentation that beleaguer efforts using cytoplasmic and membrane markers.…”

mentioning

confidence: 94%

“…Mesangial cells play a critical role in the development of glomeruli, acting in concert with podocytes and endothelial cells to form a functional filtration unit. In this issue of JASN, two papers 1,2 identify transcription factors that are necessary for mesangial cell function and consequently, glomerular tuft development.…”

mentioning

confidence: 99%

“…In the work by Grigorieva et al, 1 the authors examine the role of GATA3, a transcription factor expressed by the ureteric bud and FoxD1 + -stromal cell progenitors during development and their derivatives in adulthood. Homozygous GATA3 loss is known to cause renal agenesis in mice.…”

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confidence: 99%

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Mesangial Cells: The Tuft Guys of Glomerular Development

Marciano

2019

JASN

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3D Hydrogel Encapsulation Regulates Nephrogenesis in Kidney Organoids

Nerger,

Sinha,

Lee

et al. 2024

Advanced Materials

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Stem cell‐derived kidney organoids contain nephron segments that recapitulate morphological and functional aspects of the human kidney. However, directed differentiation protocols for kidney organoids are largely conducted using biochemical signals to control differentiation. Here, the hypothesis that mechanical signals regulate nephrogenesis is investigated in three‐dimensional culture by encapsulating kidney organoids within viscoelastic alginate hydrogels with varying rates of stress relaxation. Tubular nephron segments are significantly more convoluted in kidney organoids differentiated in encapsulating hydrogels when compared with those in suspension culture. Hydrogel viscoelasticity regulates the spatial distribution of nephron segments within the differentiating kidney organoids. Consistent with these observations, a particle‐based computational model predicts that the extent of deformation of the hydrogel‐organoid interface regulates the morphology of nephron segments. Elevated extracellular calcium levels in the culture medium, which can be impacted by the hydrogels, decrease the glomerulus‐to‐tubule ratio of nephron segments. These findings reveal that hydrogel encapsulation regulates nephron patterning and morphology and suggest that the mechanical microenvironment is an important design variable for kidney regenerative medicine.This article is protected by copyright. All rights reserved

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A Novel Role for GATA3 in Mesangial Cells in Glomerular Development and Injury

Cited by 31 publications

References 56 publications

Hypoparathyroidism, deafness, and renal dysplasia syndrome: 20 Years after the identification of the firstGATA3mutations

Hypoparathyroidism, deafness, and renal dysplasia syndrome: 20 Years after the identification of the firstGATA3mutations

Mesangial Cells: The Tuft Guys of Glomerular Development

3D Hydrogel Encapsulation Regulates Nephrogenesis in Kidney Organoids

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