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XB-ART-56118
Front Physiol January 1, 2019; 10 817.

The Many Faces of Xenopus: Xenopus laevis as a Model System to Study Wolf-Hirschhorn Syndrome.

Lasser M , Pratt B , Monahan C , Kim SW , Lowery LA .


Abstract
Wolf-Hirschhorn syndrome (WHS) is a rare developmental disorder characterized by intellectual disability and various physical malformations including craniofacial, skeletal, and cardiac defects. These phenotypes, as they involve structures that are derived from the cranial neural crest, suggest that WHS may be associated with abnormalities in neural crest cell (NCC) migration. This syndrome is linked with assorted mutations on the short arm of chromosome 4, most notably the microdeletion of a critical genomic region containing several candidate genes. However, the function of these genes during embryonic development, as well as the cellular and molecular mechanisms underlying the disorder, are still unknown. The model organism Xenopus laevis offers a number of advantages for studying WHS. With the Xenopus genome sequenced, genetic manipulation strategies can be readily designed in order to alter the dosage of the WHS candidate genes. Moreover, a variety of assays are available for use in Xenopus to examine how manipulation of WHS genes leads to changes in the development of tissue and organ systems affected in WHS. In this review article, we highlight the benefits of using X. laevis as a model system for studying human genetic disorders of development, with a focus on WHS.

PubMed ID: 31297068
PMC ID: PMC6607408
Article link: Front Physiol
Grant support: [+]

Species referenced: Xenopus laevis
Genes referenced: cplx1 ctbp1 fgfr3 fgfrl1 letm1 msx1 nelfa pigg slbp slc12a3 tacc3 twist1

Disease Ontology terms: Wolf-Hirschhorn syndrome
OMIMs: WOLF-HIRSCHHORN SYNDROME; WHS

Article Images: [+] show captions
References [+] :
Alkobtawi, Characterization of Pax3 and Sox10 transgenic Xenopus laevis embryos as tools to study neural crest development. 2019, Pubmed, Xenbase