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Frog model organisms have been appreciated for their utility in exploring physiological phenomena for nearly a century. Now, a vibrant community of biologists that utilize this model organism has poised Xenopus to serve as a high throughput vertebrate organism to model patient-driven genetic diseases. This has facilitated the investigation of effects of patient mutations on specific organs and signaling pathways. This approach promises a rapid investigation into novel mechanisms that disrupt normal organ morphology and function. Considering that many disease states are still interrogated in vitro to determine relevant biological processes for further study, the prospect of interrogating genetic disease in Xenopus in vivo is an attractive alternative. This model may more closely capture important aspects of the pathology under investigation such as cellular micro environments and local forces relevant to a specific organ's development and homeostasis. This review aims to highlight recent methodological advances that allow investigation of genetic disease in organ-specific contexts in Xenopus as well as provide examples of how these methods have led to the identification of novel mechanisms and pathways important for understanding human disease.
Figure 1. Tools in Xenopus allow for the study of heart and kidney development. Schematic of the organ systems in Xenopus along with available tools to interrogate these systems.
FIGURE 1. Tools in Xenopus allow for the study of heart and kidney development. Schematic of the organ systems in Xenopus along with available tools to interrogate these systems.
Abu-Daya,
Absence of heartbeat in the Xenopus tropicalis mutation muzak is caused by a nonsense mutation in cardiac myosin myh6.
2009, Pubmed,
Xenbase
Abu-Daya,
Absence of heartbeat in the Xenopus tropicalis mutation muzak is caused by a nonsense mutation in cardiac myosin myh6.
2009,
Pubmed
,
Xenbase Ai,
Canonical Wnt signaling functions in second heart field to promote right ventricular growth.
2007,
Pubmed Alarcón,
A dual requirement for Iroquois genes during Xenopus kidney development.
2008,
Pubmed
,
Xenbase Antin,
Precardiac mesoderm is specified during gastrulation in quail.
1994,
Pubmed Asashima,
In vitro organogenesis from undifferentiated cells in Xenopus.
2009,
Pubmed
,
Xenbase Aslan,
High-efficiency non-mosaic CRISPR-mediated knock-in and indel mutation in F0 Xenopus.
2017,
Pubmed
,
Xenbase Beyer,
Serotonin signaling is required for Wnt-dependent GRP specification and leftward flow in Xenopus.
2012,
Pubmed
,
Xenbase Bhattacharya,
CRISPR/Cas9: An inexpensive, efficient loss of function tool to screen human disease genes in Xenopus.
2015,
Pubmed
,
Xenbase Bisgrove,
The roles of cilia in developmental disorders and disease.
2006,
Pubmed Blitz,
Biallelic genome modification in F(0) Xenopus tropicalis embryos using the CRISPR/Cas system.
2013,
Pubmed
,
Xenbase Blum,
Symmetry breakage in the vertebrate embryo: when does it happen and how does it work?
2014,
Pubmed
,
Xenbase Borday,
An atlas of Wnt activity during embryogenesis in Xenopus tropicalis.
2018,
Pubmed
,
Xenbase Boskovski,
The heterotaxy gene GALNT11 glycosylates Notch to orchestrate cilia type and laterality.
2013,
Pubmed
,
Xenbase Braun,
Mutations in multiple components of the nuclear pore complex cause nephrotic syndrome.
2018,
Pubmed
,
Xenbase Buckingham,
Building the mammalian heart from two sources of myocardial cells.
2005,
Pubmed Buisson,
Pax8 and Pax2 are specifically required at different steps of Xenopus pronephros development.
2015,
Pubmed
,
Xenbase Caine,
Regeneration of functional pronephric proximal tubules after partial nephrectomy in Xenopus laevis.
2013,
Pubmed
,
Xenbase Cancer Genome Atlas Network,
Comprehensive molecular characterization of human colon and rectal cancer.
2012,
Pubmed Carroll,
Synergism between Pax-8 and lim-1 in embryonic kidney development.
1999,
Pubmed
,
Xenbase Carroll,
Dynamic patterns of gene expression in the developing pronephros of Xenopus laevis.
1999,
Pubmed
,
Xenbase Choksi,
Switching on cilia: transcriptional networks regulating ciliogenesis.
2014,
Pubmed Corkins,
Transgenic Xenopus laevis Line for In Vivo Labeling of Nephrons within the Kidney.
2018,
Pubmed
,
Xenbase DeLay,
Tissue-Specific Gene Inactivation in Xenopus laevis: Knockout of lhx1 in the Kidney with CRISPR/Cas9.
2018,
Pubmed
,
Xenbase Del Viso,
Congenital Heart Disease Genetics Uncovers Context-Dependent Organization and Function of Nucleoporins at Cilia.
2016,
Pubmed
,
Xenbase Deniz,
Analysis of Craniocardiac Malformations in Xenopus using Optical Coherence Tomography.
2017,
Pubmed
,
Xenbase Doench,
Rational design of highly active sgRNAs for CRISPR-Cas9-mediated gene inactivation.
2014,
Pubmed Dogra,
Steroid-resistant nephrotic syndrome: a persistent challenge for pediatric nephrology.
2017,
Pubmed Duncan,
Xenopus as a model organism for birth defects-Congenital heart disease and heterotaxy.
2016,
Pubmed
,
Xenbase Elkan,
The Xenopus Pregnancy Test.
1938,
Pubmed
,
Xenbase Etard,
Tracking of Indels by DEcomposition is a Simple and Effective Method to Assess Efficiency of Guide RNAs in Zebrafish.
2017,
Pubmed FOX,
ORIGIN OF THE PRONEPHRIC DUCT IN XENOPUS LAEVIS.
1964,
Pubmed
,
Xenbase Garfinkel,
An interspecies heart-to-heart: Using Xenopus to uncover the genetic basis of congenital heart disease.
2017,
Pubmed
,
Xenbase Gerdes,
The vertebrate primary cilium in development, homeostasis, and disease.
2009,
Pubmed Gessert,
The multiple phases and faces of wnt signaling during cardiac differentiation and development.
2010,
Pubmed Gessert,
Comparative gene expression analysis and fate mapping studies suggest an early segregation of cardiogenic lineages in Xenopus laevis.
2009,
Pubmed
,
Xenbase Glessner,
Increased frequency of de novo copy number variants in congenital heart disease by integrative analysis of single nucleotide polymorphism array and exome sequence data.
2014,
Pubmed Griffin,
RAPGEF5 Regulates Nuclear Translocation of β-Catenin.
2018,
Pubmed
,
Xenbase Hoff,
The nucleoside-diphosphate kinase NME3 associates with nephronophthisis proteins and is required for ciliary function during renal development.
2018,
Pubmed
,
Xenbase Homsy,
De novo mutations in congenital heart disease with neurodevelopmental and other congenital anomalies.
2015,
Pubmed Jin,
Contribution of rare inherited and de novo variants in 2,871 congenital heart disease probands.
2017,
Pubmed Kagemann,
Repeated, noninvasive, high resolution spectral domain optical coherence tomography imaging of zebrafish embryos.
2008,
Pubmed Kulkarni,
WDR5 Stabilizes Actin Architecture to Promote Multiciliated Cell Formation.
2018,
Pubmed
,
Xenbase Kwon,
Canonical Wnt signaling is a positive regulator of mammalian cardiac progenitors.
2007,
Pubmed Li,
Whole exome sequencing in 342 congenital cardiac left sided lesion cases reveals extensive genetic heterogeneity and complex inheritance patterns.
2017,
Pubmed Ma,
Ciliary Mechanisms of Cyst Formation in Polycystic Kidney Disease.
2017,
Pubmed Manheimer,
Robust identification of mosaic variants in congenital heart disease.
2018,
Pubmed Marvin,
Inhibition of Wnt activity induces heart formation from posterior mesoderm.
2001,
Pubmed
,
Xenbase Mitchison,
Motile and non-motile cilia in human pathology: from function to phenotypes.
2017,
Pubmed Moody,
Fates of the blastomeres of the 16-cell stage Xenopus embryo.
1987,
Pubmed
,
Xenbase Moreno-Mateos,
CRISPRscan: designing highly efficient sgRNAs for CRISPR-Cas9 targeting in vivo.
2015,
Pubmed
,
Xenbase Morin,
Activation of beta-catenin-Tcf signaling in colon cancer by mutations in beta-catenin or APC.
1997,
Pubmed Moriya,
Induction of Pronephric Tubules by Activin and Retinoic Acid in Presumptive Ectoderm of Xenopus laevis: (RA/kidney/mesoderm induction/Xenopus laevis).
1993,
Pubmed
,
Xenbase Nachury,
Establishing and regulating the composition of cilia for signal transduction.
2019,
Pubmed Naito,
Developmental stage-specific biphasic roles of Wnt/beta-catenin signaling in cardiomyogenesis and hematopoiesis.
2006,
Pubmed Nakamura,
A Wnt- and beta -catenin-dependent pathway for mammalian cardiac myogenesis.
2003,
Pubmed Nascone,
Organizer induction determines left-right asymmetry in Xenopus.
1997,
Pubmed
,
Xenbase Nobes,
Rho, rac and cdc42 GTPases: regulators of actin structures, cell adhesion and motility.
1995,
Pubmed Oh,
Cilia in vertebrate development and disease.
2012,
Pubmed Osafune,
In vitro induction of the pronephric duct in Xenopus explants.
2002,
Pubmed
,
Xenbase Raciti,
Organization of the pronephric kidney revealed by large-scale gene expression mapping.
2008,
Pubmed
,
Xenbase Rodriguez,
Congenital Anomalies of the Kidney and the Urinary Tract (CAKUT).
2014,
Pubmed Sater,
The specification of heart mesoderm occurs during gastrulation in Xenopus laevis.
1989,
Pubmed
,
Xenbase Saulnier,
Essential function of Wnt-4 for tubulogenesis in the Xenopus pronephric kidney.
2002,
Pubmed
,
Xenbase Schneider,
Wnt antagonism initiates cardiogenesis in Xenopus laevis.
2001,
Pubmed
,
Xenbase Sifrim,
Distinct genetic architectures for syndromic and nonsyndromic congenital heart defects identified by exome sequencing.
2016,
Pubmed Smith,
The MLC1v gene provides a transgenic marker of myocardium formation within developing chambers of the Xenopus heart.
2005,
Pubmed
,
Xenbase Smith,
Early cardiac morphogenesis defects caused by loss of embryonic macrophage function in Xenopus.
2011,
Pubmed
,
Xenbase Smith,
Mesoderm-inducing factors and the control of gastrulation.
1992,
Pubmed
,
Xenbase Sparrow,
Regulation of the tinman homologues in Xenopus embryos.
2000,
Pubmed
,
Xenbase Tran,
Design and use of transgenic reporter strains for detecting activity of signaling pathways in Xenopus.
2014,
Pubmed
,
Xenbase Tran,
Wnt/beta-catenin signaling is involved in the induction and maintenance of primitive hematopoiesis in the vertebrate embryo.
2010,
Pubmed
,
Xenbase Triedman,
Trends in Congenital Heart Disease: The Next Decade.
2016,
Pubmed Tzahor,
Wnt/beta-catenin signaling and cardiogenesis: timing does matter.
2007,
Pubmed Ueno,
Biphasic role for Wnt/beta-catenin signaling in cardiac specification in zebrafish and embryonic stem cells.
2007,
Pubmed van der Linde,
Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis.
2011,
Pubmed Vivante,
A Dominant Mutation in Nuclear Receptor Interacting Protein 1 Causes Urinary Tract Malformations via Dysregulation of Retinoic Acid Signaling.
2017,
Pubmed
,
Xenbase Vize,
Development of the Xenopus pronephric system.
1995,
Pubmed
,
Xenbase Walentek,
ATP4a is required for Wnt-dependent Foxj1 expression and leftward flow in Xenopus left-right development.
2012,
Pubmed
,
Xenbase Wallingford,
Strange as it may seem: the many links between Wnt signaling, planar cell polarity, and cilia.
2011,
Pubmed
,
Xenbase Werner,
Using Xenopus skin to study cilia development and function.
2013,
Pubmed
,
Xenbase Wild,
The mutated human gene encoding hepatocyte nuclear factor 1beta inhibits kidney formation in developing Xenopus embryos.
2000,
Pubmed
,
Xenbase Yoshiba,
Roles of cilia, fluid flow, and Ca2+ signaling in breaking of left-right symmetry.
2014,
Pubmed Zaidi,
De novo mutations in histone-modifying genes in congenital heart disease.
2013,
Pubmed Zhou,
Proximo-distal specialization of epithelial transport processes within the Xenopus pronephric kidney tubules.
2004,
Pubmed
,
Xenbase
