Ectodysplasin signaling in development.
Abstract
Ectodysplasin (Eda), a signaling molecule belonging to the tumor necrosis factor family, is required for normal development of several ectodermally derived organs in humans and mice. Two closely related isoforms of ectodysplasin, Eda-A1 and Eda-A2, have been described which bind to and activate two different receptors, Edar and X-linked Eda-A2 receptor (Xedar), respectively. Mutations in Eda, Edar or other molecules of this signaling pathway cause ectodermal dysplasias characterized by defective development of teeth, hairs, and several exocrine glands such as sweat glands presumably due to impaired NF-kappaB response. Studies with mice either lacking the functional proteins of Edar pathway or overexpressing the ligand or receptor suggest that Eda-A1-Edar signaling has multiple roles in ectodermal organ development regulating their initiation, morphogenesis, and differentiation.
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In mammals, the EDA signaling pathway is involved in hair and teeth formation, and mutations affecting this pathway cause human HED.
“In mammals the EDA signaling pathway is involved in hair and teeth formation [9] and mutations affecting this pathway cause the human hereditary disease hypohidrotic ectodermal dysplasia (HED).”
From Zebrafish eda and edar Mutants Reveal Conserved and Ancestral Roles of Ectodysplasin Signaling in Vertebrates (2008) · cites this paper as [9]
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- Authors
- Mikkola, M. L., & Thesleff, I.
- Affiliations
- University of Helsinki (FI)
- Corresponding author
- Mikkola, Marja L.
- Venue
- Cytokine & Growth Factor Reviews
- Publisher
- Elsevier BV
- Published
- 2003-06
- Volume
- 14
- Issue
- 3-4
- Pages
- 211-224
- ISSN
- 1359-6101 (ISSN-L)
- OpenAlex
- W2020600328
- Cited by
- 217
- References
- 7
- Citations by year
- 2026: 5 · 2025: 4 · 2024: 9 · 2023: 6 · 2022: 11 · 2021: 8 · 2020: 7 · 2019: 11 · 2018: 7 · 2017: 4 · 2016: 8 · 2015: 7 · 2014: 11 · 2013: 9 · 2012: 7
- FWCI
- 1.02
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Cytokine & Growth Factor Reviews (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › dental development and anomalies
- Health Sciences › Medicine › Urology › Hair Growth and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Developmental Biology and Gene Regulation
- Keywords
- ectodysplasin, tumor necrosis factor family, ectodermal dysplasia, tooth development, hair development, sweat gland development, NF-κB signaling, morphogenesis
- MeSH terms
- Animals, Embryonic and Fetal Development, Humans, Membrane Proteins, Models, Biological, Mutation, Signal Transduction, Receptors, Tumor Necrosis Factor, Gene Expression Regulation, Developmental, Protein Isoforms, Mice, Ectodysplasins, Receptors, Ectodysplasin, Edar Receptor, Xedar Receptor
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