Lesions in the anhidrotic ectodermal dysplasia (EDA) gene cause the recessive human genetic disorder X-linked anhidrotic ectodermal dysplasia, which is characterized by the poor development of ectoderm-derived structures. Ectodysplasin-A, the protein encoded by the EDA gene, is a member of the tumor necrosis factor ligand superfamily that forms a collagen triple helix, suggesting functions in signal transduction and cell adhesion. In an effort to elucidate the function of EDA in pathways regulating ectodermal development, we have analyzed promoter elements of the gene. We show here that a binding site for the lymphocyte enhancer factor 1 (Lef-1) transcription factor is active. In electrophoretic mobility shift assays, Lef-1 specifically bound to its site in the EDA promoter. Over-expression of both Lef-1 and beta-catenin significantly increased EDA transcription in co-transfection studies. In addition, indirect stabilization of endogenous beta-catenin stimulated EDA transcription 4- to 13-fold. This is the first direct evidence of a relationship between EDA and the Wnt pathway. We have also investigated whether EDA might function in a feedback loop to modulate Wnt signaling. Over-expression of EDA neither stimulated basal transcription of Wnt-dependent genes, nor inhibited Wnt-dependent activation of transcription. Taken together, our results demonstrate that Wnt signaling does control EDA gene expression, but ectodysplasin-A does not feedback on the Wnt pathway.
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http://dx.doi.org/10.1016/s0378-1119(02)00407-9 | DOI Listing |
J Clin Med
January 2025
Department of Systems Medicine, University of Rome Tor Vergata, 00133 Rome, Italy.
: The nuclear factor (NF)-kB essential modulator (NEMO) has a crucial role in the NFκB pathway. Hypomorphic pathogenic variants cause ectodermal dysplasia with immunodeficiency (EDA-ID) in affected males. However, heterozygous amorphic variants could be responsible for Incontinentia Pigmenti (IP) in female carriers.
View Article and Find Full Text PDFGenes (Basel)
December 2024
Department of Pediatric Dentistry & DRI, School of Dentistry, Seoul National University, Seoul 03080, Republic of Korea.
Background/objectives: The ectodysplasin A () gene, a member of the tumor necrosis factor ligand superfamily, is involved in the early epithelial-mesenchymal interaction that regulates ectoderm-derived appendage formation. Numerous studies have shown that mutations in the gene can cause X-linked ectodermal dysplasia (ED) and non-syndromic oligodontia (NSO). Accordingly, this study aimed to identify the causative genetic mutations of the gene.
View Article and Find Full Text PDFCells
January 2025
Department of Cellular Pathology, Institute for Developmental Research, Aichi Developmental Disability Center, Kasugai 480-0392, Aichi, Japan.
Dendritic spine formation/maintenance is highly dependent on actin cytoskeletal dynamics, which is regulated by small GTPases Rac1 and Cdc42 through their downstream p21-activated kinase/LIM-kinase-I/cofilin pathway. ARHGEF7, also known as ß-PIX, is a guanine nucleotide exchange factor for Rac1 and Cdc42, thereby activating Rac1/Cdc42 and the downstream pathway, leading to the upregulation of spine formation/maintenance. We found that STIL, one of the primary microcephaly gene products, is associated with ARHGEF7 in dendritic spines and that knockdown of resulted in a significant reduction in dendritic spines in neurons both in vitro and in vivo.
View Article and Find Full Text PDFSci Rep
January 2025
College of Life and Environmental Sciences, University of Exeter, Biosciences, Exeter, EX4 4QD, UK.
The mangrove killifish, Kryptolebias marmoratus, can reproduce with self-fertilisation, offering a unique and useful genetic tool for generation of genetic mutants and quick identification of mutated genes. From an ENU-mutated mangrove killifish line R228, we have isolated a novel mutant line, no-fin-ray/nfr in which homozygous mutant of adult fish fin ray development is largely reduced. Illumina RNAseq with 3 embryos each from mutants, siblings and the parental WT strain Hon9 (only 9 embryos as total) identified a mutation in the edaradd in a highly conserved C-terminal death domain.
View Article and Find Full Text PDFACS Chem Neurosci
January 2025
Department of Neurology, Multi-Omics Research Center for Brain Disorders,The First Affiliated Hospital, Hengyang Medical School, University of South China, Hengyang, Hunan 421001, China.
Brachial plexus root avulsion (BPRA) is often caused by road collisions, leading to total loss of motor function in the upper limb. At present, effective treatment options remain limited. Edaravone (EDA), a substance that eliminates free radicals, exhibits numerous biological properties, including neuroprotective, antioxidant and anti-inflammatory effects.
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