Expression patterns of the Wtx/Amer gene family during mouse embryonic development

Abstract

WTX/AMER1 is a novel negative regulator of the WNT/b-catenin pathway with mutations detected in Wilms' tumors and an X-linked sclerosing bone dysplasia. WTX/AMER1 (Fam123b) shares several domains of homology with two other recently identified proteins: AMER2 (Fam123a) and AMER3 (Fam123c). Here, we describe an in-depth expression analysis of all three members of this gene family during mouse embryonic development. All genes were strongly expressed in the central as well as the peripheral nervous system, thus suggesting important roles of this gene family during neurogenesis. Specific expression was found in the retina, inner ear, and nasal epithelium. Outside of the nervous system Wtx/Amer1 showed the broadest expression domains including cephalic and limb mesenchyme, skeletal muscle, bladder, gonads, lung bud, salivary glands, and the kidneys. The widespread expression pattern of Wtx/Amer1, together with its role as a modulator of the Wnt signaling pathway, suggest that Wtx/Amer1 serves pleiotropic roles during mammalian organogenesis.

Gain of <b><i>FAM123B</i></b> and <b><i>ARHGEF9 </i></b>in an Obese Man with Intellectual Disability, Congenital Heart Defects and Multiple Supernumerary Ring Chromosomes

“…The gene encodes a component of the ␤ -catenin degradation complex that inhibits WNT signaling and may function in the transcriptional regulation of cellular differentiation [Rivera et al, 2009]. During mouse embryogenesis, the gene is strongly transcribed in the central and peripheral nervous system [Comai et al, 2010]. These observations support a role in intellectual disability as seen in our patient.…”

Amer2 Protein Is a Novel Negative Regulator of Wnt/β-Catenin Signaling Involved in Neuroectodermal Patterning

“…However, the two proteins have overlapping but distinct expression profiles in the mouse, and a recent knockout study showed that Amer1 is critical for differentiation of mesenchymal progenitor cells (9,30), whereas our Xenopus experiments indicate that Amer2 is relevant for development of neural tissues, in line with its restricted expression pattern. Thus, Amer proteins appear to have tissue-specific functional roles.…”

“…Amer1 is mutated in Wilms tumors and in the inherited disease osteopathia striata congenita with cranial sclerosis (7,8). A recent expression analysis of the Amer gene family in the mouse showed rather widespread expression of Amer1, whereas Amer2 and, even more so, Amer3 were largely restricted to the nervous system (9). Apart from its APC-binding activity little is known about the regulation and function of Amer2.…”

The adrenal capsule is a signaling center controlling cell renewal and zonation through Rspo3

“…The Gli1 in situ probe was kindly provided by M. Studer (Institute of Biology Valrose, Université de NiceSophia Antipolis). Seven-micrometer-thick sections were hybridized with Rspo1, Rspo3, Wnt4, and Shh as well as Gli1 probes according to previously described protocols (Comai et al 2010). RNA-Scope analysis for Rspo3 was performed on adult sections according to the manufacturer's instructions.…”