Reduced Pax2 gene dosage increases apoptosis and slows the progression of renal cystic disease.

Abstract:

:The murine cpk mouse develops a rapid-onset polycystic kidney disease (PKD) with many similarities to human PKD. During kidney development, the transcription factor Pax2 is required for the specification and differentiation of the renal epithelium. In humans, Pax2 is also expressed in juvenile cystic kidneys where it correlates with cell proliferation. In this report, Pax2 expression is demonstrated in the cystic epithelium of the mouse cpk kidneys. To assess the role of Pax2 during the development of polycystic kidney disease, the progression of renal cysts was examined in cpk mutants carrying one or two alleles of Pax2. Reduced Pax2 gene dosage resulted in a significant inhibition of renal cyst growth while maintaining more normal renal structures. The inhibition of cyst growth was not due to reduced proliferation of the cystic epithelium, rather to increased cell death in the Pax2 heterozygotes. Increased apoptosis with reduced Pax2 gene dosage was also observed in normal developing kidneys. Thus, increased cell death is an integral part of the Pax2 heterozygous phenotype and may be the underlying cause of Pax gene haploinsufficiency. That the cystic epithelium requires Pax2 for continued expansion underscores the embryonic nature of the renal cystic cells and may provide new insights toward growth suppression strategies.

journal_name

Dev Biol

journal_title

Developmental biology

authors

Ostrom L,Tang MJ,Gruss P,Dressler GR

doi

10.1006/dbio.2000.9618

subject

Has Abstract

pub_date

2000-03-15 00:00:00

pages

250-8

issue

2

eissn

0012-1606

issn

1095-564X

pii

S0012-1606(00)99618-0

journal_volume

219

pub_type

杂志文章
  • Timing and rates of synthesis of early histone mRNA in the embryo of Strongylocentrotus purpuratus.

    abstract::The level of early histone mRNA in Strongylocentrotus purpuratus changes abruptly at 6 hr of development, increasing an average of 10-fold by 9-10.5 hr and then decreasing over 2-fold by 13.5-15 hr. These changes occur when the late embryonic mRNA is still a very minor component of histone gene transcription. The exac...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/0012-1606(83)90340-8

    authors: Weinberg ES,Hendricks MB,Hemminki K,Kuwabara PE,Farrelly LA

    更新日期:1983-07-01 00:00:00

  • Epidermal growth factor inhibits morphogenesis and cell differentiation in cultured mouse embryonic teeth.

    abstract::Although local epithelial-mesenchymal tissue interactions which are presumably mediated by extracellular matrix molecules are important regulators of tooth morphogenesis and differentiation, our studies have indicated that these developmental processes also depend on circulating molecules. The iron-carrying serum prot...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/0012-1606(85)90437-3

    authors: Partanen AM,Ekblom P,Thesleff I

    更新日期:1985-09-01 00:00:00

  • Eukaryotic initiation factor eIF6 modulates the expression of Kermit 2/XGIPC in IGF- regulated eye development.

    abstract::The eukaryotic initiation translation factor eIF6 is a highly conserved, essential protein implicated in translation. eIF6 is regulated in vivo by extracellular signals, such as IGF signaling (for a review see Miluzio et al., 2009). In Xenopus, eif6 over-expression causes a delay in eye development (De Marco et al., 2...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2017.04.017

    authors: De Marco N,Tussellino M,Carotenuto R,Ronca R,Rizzolio S,Biffo S,Campanella C

    更新日期:2017-07-01 00:00:00

  • Activin signals via SMAD2/3 between germ and somatic cells in the human fetal ovary and regulates kit ligand expression.

    abstract::Ovarian germ cell survival is dependent upon the formation of primordial follicles, which occurs during fetal life in the human. Activin contributes to germ cell proliferation and survival at this time. SMADs2 and 3 are central elements in the activin signalling pathway and thus indicate sites of activin action. We ha...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2007.11.026

    authors: Coutts SM,Childs AJ,Fulton N,Collins C,Bayne RA,McNeilly AS,Anderson RA

    更新日期:2008-02-01 00:00:00

  • BMP receptor IA is required in the mammalian embryo for endodermal morphogenesis and ectodermal patterning.

    abstract::BMPRIA is a receptor for bone morphogenetic proteins with high affinity for BMP2 and BMP4. Mouse embryos lacking Bmpr1a fail to gastrulate, complicating studies on the requirements for BMP signaling in germ layer development. Recent work shows that BMP4 produced in extraembryonic tissues initiates gastrulation. Here w...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2004.01.048

    authors: Davis S,Miura S,Hill C,Mishina Y,Klingensmith J

    更新日期:2004-06-01 00:00:00

  • Precocious retinal neurons: Pax6 controls timing of differentiation and determination of cell type.

    abstract::The transcription factor Pax6 plays a pivotal role in eye development, as eye morphogenesis is arrested at a primitive optic vesicle stage in homozygous Pax6 mutant mouse embryos. The arrested optic vesicle development has led to the assumption that cellular differentiation programs are unable to initiate. Contrary to...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2004.12.018

    authors: Philips GT,Stair CN,Young Lee H,Wroblewski E,Berberoglu MA,Brown NL,Mastick GS

    更新日期:2005-03-15 00:00:00

  • Mechanisms of mesendoderm internalization in the Xenopus gastrula: lessons from the ventral side.

    abstract::Two main processes are involved in driving ventral mesendoderm internalization in the Xenopus gastrula. First, vegetal rotation, an active movement of the vegetal cell mass, initiates gastrulation by rolling the peripheral blastocoel floor against the blastocoel roof. In this way, the leading edge of the internalized ...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1006/dbio.2001.0459

    authors: Ibrahim H,Winklbauer R

    更新日期:2001-12-01 00:00:00

  • Multi-scale regulation of cell branching: Modeling morphogenesis.

    abstract::Plant growth and development are driven by extended phases of irreversible cell expansion generating cells that increase in volume from 10- to 100-fold. Some specialized cell types define cortical sites that reinitiate polarized growth and generate branched cell morphology. This structural specialization of individual...

    journal_title:Developmental biology

    pub_type: 杂志文章,评审

    doi:10.1016/j.ydbio.2018.12.004

    authors: Li J,Kim T,Szymanski DB

    更新日期:2019-07-01 00:00:00

  • Evolutionary developmental transition from median to paired morphology of vertebrate fins: Perspectives from twin-tail goldfish.

    abstract::Vertebrate morphology has been evolutionarily modified by natural and/or artificial selection. The morphological variation of goldfish is a representative example. In particular, the twin-tail strain of ornamental goldfish shows highly diverged anal and caudal fin morphology: bifurcated anal and caudal fins. Recent mo...

    journal_title:Developmental biology

    pub_type: 杂志文章,评审

    doi:10.1016/j.ydbio.2016.11.022

    authors: Abe G,Ota KG

    更新日期:2017-07-15 00:00:00

  • TFE2 and GATA3 enhance induction of POU4F3 and myosin VIIa positive cells in nonsensory cochlear epithelium by ATOH1.

    abstract::Transcription factors (TFs) can regulate different sets of genes to determine specific cell types by means of combinatorial codes. We previously identified closely-spaced TF binding motifs located 8.2-8.5 kb 5' to the ATG of the murine Pou4f3 gene, a gene required for late hair cell (HC) differentiation and survival. ...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2012.09.002

    authors: Masuda M,Pak K,Chavez E,Ryan AF

    更新日期:2012-12-01 00:00:00

  • The Caenorhabditis elegans AHR-1 transcription complex controls expression of soluble guanylate cyclase genes in the URX neurons and regulates aggregation behavior.

    abstract::C. elegans ahr-1 is orthologous to the mammalian aryl hydrocarbon receptor, and it functions as a transcription factor to regulate the development of certain neurons. Here, we describe the role of ahr-1 in a specific behavior: the aggregation of C. elegans on lawns of bacterial food. This behavior is modulated by nutr...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2006.07.017

    authors: Qin H,Zhai Z,Powell-Coffman JA

    更新日期:2006-10-15 00:00:00

  • An overlapping CArG/octamer element is required for regulation of desmin gene transcription in arterial smooth muscle cells.

    abstract::The desmin gene encodes an intermediate filament protein that is present in skeletal, cardiac, and smooth muscle cells. This study shows that the 4-kb upstream region of the murine desmin promoter directs expression of a lacZ reporter gene throughout the heart from E7.5 and in skeletal muscle and vascular smooth muscl...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1006/dbio.2000.9865

    authors: Mericskay M,Parlakian A,Porteu A,Dandré F,Bonnet J,Paulin D,Li Z

    更新日期:2000-10-15 00:00:00

  • Teloplasm formation in a leech, Helobdella triserialis, is a microtubule-dependent process.

    abstract::Fertilized eggs of the leech Helobdella triserialis undergo a cytoplasmic reorganization which generates domains of nonyolky cytoplasm, called teloplasm, at the animal and vegetal poles. The segregation of teloplasm to one cell of the eight-cell embryo is responsible for a unique developmental fate of that cell, i.e.,...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/0012-1606(89)90181-4

    authors: Astrow SH,Holton B,Weisblat DA

    更新日期:1989-10-01 00:00:00

  • Expression of pluripotency factors in larval epithelia of the frog Xenopus: evidence for the presence of cornea epithelial stem cells.

    abstract::Understanding the biology of somatic stem cells in self renewing tissues represents an exciting field of study, especially given the potential to harness these cells for tissue regeneration and repair in treating injury and disease. The mammalian cornea contains a population of basal epithelial stem cells involved in ...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2012.12.005

    authors: Perry KJ,Thomas AG,Henry JJ

    更新日期:2013-02-15 00:00:00

  • Epithelial sodium conductance in rabbit preimplantation trophectodermal cells.

    abstract::We examined the development of epithelial Na+ conductance in 6- and 7-day post coitus (p.c.) preimplantation rabbit embryos using the whole-cell patch-clamp technique on dissociated rabbit trophectodermal cells and by immunocytochemical localization using a polyclonal antibody directed against subunits of an apical ep...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/0012-1606(91)90289-f

    authors: Robinson DH,Bubien JK,Smith PR,Benos DJ

    更新日期:1991-10-01 00:00:00

  • A large-scale RNAi screen identifies functional classes of genes shaping synaptic development and maintenance.

    abstract::Neuronal circuit development and function require proper synapse formation and maintenance. Genetic screens are one powerful method to identify the mechanisms shaping synaptic development and stability. However, genes with essential roles in non-neural tissues may be missed in traditional loss-of-function screens. In ...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2012.04.008

    authors: Valakh V,Naylor SA,Berns DS,DiAntonio A

    更新日期:2012-06-15 00:00:00

  • Development of the enteric nervous system in the moth. I. Diversity of cell types and the embryonic expression of FMRFamide-related neuropeptides.

    abstract::The enteric nervous system (ENS) of the larval moth Manduca sexta consists of two small ganglia and several nerve networks that lie superficially along the alimentary tract. Within this system are approximately 600 neurons that exhibit a spectrum of biochemical and morphological characteristics and that express these ...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/s0012-1606(89)80039-9

    authors: Copenhaver PF,Taghert PH

    更新日期:1989-01-01 00:00:00

  • Dynamic phosphorylation of the kinesin Costal-2 in vivo reveals requirement of fused kinase activity for all levels of hedgehog signalling.

    abstract::Hedgehog (Hh) proteins are secreted molecules that play an essential role in development and tumorigenesis. In Drosophila cultured cells, phosphorylation of the kinesin-like Costal2 (Cos2) protein at Ser572 is triggered by the kinase fused (Fu) upon Hh pathway activation. Here, we validate the first phospho-antibody f...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2010.04.021

    authors: Raisin S,Ruel L,Ranieri N,Staccini-Lavenant L,Thérond PP

    更新日期:2010-08-01 00:00:00

  • Topographic targeting and pathfinding errors of retinal axons following overexpression of ephrinA ligands on retinal ganglion cell axons.

    abstract::In the retinotectal projection, the Eph receptor tyrosine kinase ligands ephrinA2 and ephrinA5 are differentially expressed not only in the tectum, but also in a high-nasal-to-low-temporal pattern in the retina. Recently, we have shown that retrovirally driven overexpression of ephrinA2 on retinal axons leads to topog...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1006/dbio.1999.9489

    authors: Dütting D,Handwerker C,Drescher U

    更新日期:1999-12-01 00:00:00

  • Neural development in Onychophora (velvet worms) suggests a step-wise evolution of segmentation in the nervous system of Panarthropoda.

    abstract::A fundamental question in biology is how animal segmentation arose during evolution. One particular challenge is to clarify whether segmental ganglia of the nervous system evolved once, twice, or several times within the Bilateria. As close relatives of arthropods, Onychophora play an important role in this debate sin...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2009.08.011

    authors: Mayer G,Whitington PM

    更新日期:2009-11-01 00:00:00

  • FGF-10 plays an essential role in the growth of the fetal prostate.

    abstract::Induction and branching morphogenesis of the prostate are dependent on androgens, which act via the mesenchyme to induce prostatic epithelial development. One mechanism by which the mesenchyme may regulate the epithelium is through secreted growth factors such as FGF-10. We have examined the male reproductive tract of...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/s0012-1606(03)00250-1

    authors: Donjacour AA,Thomson AA,Cunha GR

    更新日期:2003-09-01 00:00:00

  • Xenopus laevis zygote arrest 2 (zar2) encodes a zinc finger RNA-binding protein that binds to the translational control sequence in the maternal Wee1 mRNA and regulates translation.

    abstract::Zygote arrest (Zar) proteins are crucial for early embryonic development, but their molecular mechanism of action is unknown. The Translational Control Sequence (TCS) in the 3' untranslated region (UTR) of the maternal mRNA, Wee1, mediates translational repression in immature Xenopus oocytes and translational activati...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2012.06.012

    authors: Charlesworth A,Yamamoto TM,Cook JM,Silva KD,Kotter CV,Carter GS,Holt JW,Lavender HF,MacNicol AM,Ying Wang Y,Wilczynska A

    更新日期:2012-09-15 00:00:00

  • Morphogenesis and motility of the Astyanax mexicanus gastrointestinal tract.

    abstract::Through the course of evolution, the gastrointestinal (GI) tract has been modified to maximize nutrient absorption, forming specialized segments that are morphologically and functionally distinct. Here we show that the GI tract of the Mexican tetra, Astyanax mexicanus, has distinct regions, exhibiting differences in m...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2018.06.004

    authors: Riddle MR,Boesmans W,Caballero O,Kazwiny Y,Tabin CJ

    更新日期:2018-09-15 00:00:00

  • Heterochrony and the phylotypic period.

    abstract::There has been a resurgence of interest in comparative embryology. It is now important to be able to compare gene expression in different species at similar developmental stages. One phenomenon which may make it difficult to compare embryos in this way is heterochrony--a change in developmental timing during evolution...

    journal_title:Developmental biology

    pub_type: 杂志文章,评审

    doi:10.1006/dbio.1995.8041

    authors: Richardson MK

    更新日期:1995-12-01 00:00:00

  • Identification of satellite cells from anole lizard skeletal muscle and demonstration of expanded musculoskeletal potential.

    abstract::The lizards are evolutionarily the closest vertebrates to humans that demonstrate the ability to regenerate entire appendages containing cartilage, muscle, skin, and nervous tissue. We previously isolated PAX7-positive cells from muscle of the green anole lizard, Anolis carolinensis, that can differentiate into multin...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2017.08.037

    authors: Palade J,Djordjevic D,Hutchins ED,George RM,Cornelius JA,Rawls A,Ho JWK,Kusumi K,Wilson-Rawls J

    更新日期:2018-01-15 00:00:00

  • Suppressor of Hairless and Presenilin phenotypes imply involvement of canonical Notch-signalling in segmentation of the spider Cupiennius salei.

    abstract::Arthropods, vertebrates, and annelids all have a segmented body. Our recent discovery of involvement of Notch-signalling in spider segmentation revived the discussion on the origin of segmented body plans and suggests the sharing of a common genetic program in a common ancestor. Here, we analysed the spider homologues...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2005.01.024

    authors: Schoppmeier M,Damen WG

    更新日期:2005-04-01 00:00:00

  • The 5' UTR and 3' UTR of the sperm mitochondria-associated cysteine-rich protein mRNA regulate translation in spermatids by multiple mechanisms in transgenic mice.

    abstract::The Smcp mRNA encoding the sperm mitochondria-associated cysteine-rich protein is translationally repressed in round spermatids and translationally active in elongated spermatids. The patterns of transcription and translation of fusions of the Smcp promoter, the green fluorescent protein coding region (Gfp) and variou...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2006.04.468

    authors: Hawthorne SK,Busanelli RR,Kleene KC

    更新日期:2006-09-01 00:00:00

  • Par-1 and Tau regulate the anterior-posterior gradient of microtubules in Drosophila oocytes.

    abstract::The formation of an anterior-posterior (AP) gradient of microtubules in Drosophila oocytes is essential for specification of the AP axis. Proper microtubule organization in the oocyte requires the function of serine/threonine kinase Par-1. The N1S isoform of Par-1 is enriched at the posterior cortex of the oocyte from...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1016/j.ydbio.2008.12.031

    authors: Tian AG,Deng WM

    更新日期:2009-03-15 00:00:00

  • SpRunt-1, a new member of the runt domain family of transcription factors, is a positive regulator of the aboral ectoderm-specific CyIIIA gene in sea urchin embryos.

    abstract::In this paper we present a structural and functional characterization of a new sea urchin embryo transcription factor, SpRunt-1. This factor was isolated by means of its specific interaction with a cis-regulatory target site of the CyIIIa gene. Here we show that this target site, the P7I site, is required for normal e...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1006/dbio.1996.0050

    authors: Coffman JA,Kirchhamer CV,Harrington MG,Davidson EH

    更新日期:1996-02-25 00:00:00

  • Enhanced expression of limb malformations and axial skeleton alterations in legless mutants by transplacental exposure to retinoic acid.

    abstract::This manuscript reports on the limb malformations and axial skeleton alterations found in legless fetuses and their heterozygote and wild-type littermates transplacentally exposed to all-trans-retinoic acid via a single intraperitoneal injection on Day 7, 8, 9, 9.5, 10, 10.5, or 11 of gestation. The most surprising as...

    journal_title:Developmental biology

    pub_type: 杂志文章

    doi:10.1006/dbio.1994.1198

    authors: Scott WJ,Collins MD,Ernst AN,Supp DM,Potter SS

    更新日期:1994-07-01 00:00:00