Arid
DOI10.1002/ajmg.a.20495
The Hedgehog signaling network
Cohen, MM
通讯作者Cohen, MM
来源期刊AMERICAN JOURNAL OF MEDICAL GENETICS PART A
ISSN0148-7299
出版年2003
期号1页码:5-28
英文摘要

In the hedgehog signaling network, mutations result in various phenotypes, including, among others, holoprosencephaly, nevoid basal cell carcinoma syndrome, Pallister-Hall syndrome, Greig cephalopolysyndactyly, Rubinstein-Taybi syndrome, isolated basal cell carcinoma, and medulloblastoma. Active Hedgehog ligand is double lipid modified with a C-terminal cholesterol moiety and an N-terminal palmitate. Transport active Hedgehog from the signaling cell to the responding cell occurs through three mechanisms: 1). formation of multimeric Hedgehog which makes it soluble; 2). function of Dispatched in releasing the lipid-anchored protein from the signaling cell; and 3). movement across the plasma membrane of the responding cell by Tout-velu-dependent synthesis of heparan sulfate proteoglycan. In the responding cell, active Hedgehog binds to its receptor Patched, a 12-pass transmembrane protein, which frees Smoothened, an adjacent 7-pass transmembrane protein, for downstream signaling. Patched and Smoothened may shuttle oppositely between the plasma membrane and endocytic vesicles in response to active Hedgehog ligand. In downstream signaling, Cubitus interruptus (Gli proteins in vertebrates), Costal 2, Fused, and Suppressor of Fused form a tetrameric complex. Cubitus interruptus is a bifunctional transcription regulator. In the absence of active Hedgehog ligand, a truncated transcriptional repressor is generated that binds target genes and blocks their transcription. In the presence of active Hedgehog ligand, a full length transcriptional activator binds target genes and upregulates their transcription. Target genes include Wingless (Wnt gene family in vertebrates), Decapentaplegic (Bone Morphogenetic Proteins in vertebrates), and Patched. The upregulation of Patched expression, resulting in Patched protein at the cell membrane, sequesters Hedgehog and limits its spread beyond the cells in which it is produced. Thus, a balance is created by the antagonism of Hedgehog and Patched, whose relative concentrations alternate with respect to each other. Many more factors that are essential for the hedgehog signaling network are also discussed: Megalin, Rab23, Hip, GAS1, PKA, GSK3, CK1, Slimb, SAP18, and CBP. (C) 2003 Wiley-Liss, Inc.


英文关键词Sonic Hedgehog Indian Hedgehog Desert Hedgehog Multimeric Hedgehog cholesterol metabolism palmitoylation Dispatched Hip GAS1 Tout-velu EXT gene family Patched Patched 2 TRC8 sterol sensing domain Megalin Rab23 Smoothened Cubitus interruptus Gli gene family Costal 2 Fused Suppressor of Fused PKA Slimb SAP18 GSK3 CK1 CBP Decapentaplegic BMPs Wingless Wnt gene family holoprosencephaly nevoid basal cell carcinoma syndrome Pallister-Hall syndrome Greig cephalopolysyndactyly Rubinstein-Taybi syndrome multiple exostoses VACTERL
类型Article ; Proceedings Paper
语种英语
国家Canada
收录类别CPCI-S ; SCI-E
WOS记录号WOS:000186239400002
WOS关键词BASAL-CELL CARCINOMA ; STEROL-SENSING DOMAIN ; SEGMENT POLARITY GENE ; PRIMITIVE NEUROECTODERMAL TUMORS ; VERATRUM-CALIFORNICUM DURAND ; RUBINSTEIN-TAYBI-SYNDROME ; CENTRAL-NERVOUS-SYSTEM ; LEMLI-OPITZ-SYNDROME ; DROSOPHILA PATCHED GENE ; IN-SITU HYBRIDIZATION
WOS类目Genetics & Heredity
WOS研究方向Genetics & Heredity
资源类型期刊论文
条目标识符http://119.78.100.177/qdio/handle/2XILL650/144048
作者单位(1)Dalhousie Univ, Dept Oral & Maxillofacial Sci, Halifax, NS B3H 3J5, Canada;(2)Dalhousie Univ, Dept Pediat, Halifax, NS B3H 3J5, Canada;(3)Dalhousie Univ, Dept Community Hlth & Epidemiol, Halifax, NS B3H 3J5, Canada;(4)Dalhousie Univ, Dept Hlth Sci Adm, Halifax, NS B3H 3J5, Canada;(5)Dalhousie Univ, Dept Sociol & Social Anthropol, Halifax, NS B3H 3J5, Canada
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Cohen, MM. The Hedgehog signaling network[J],2003(1):5-28.
APA Cohen, MM.(2003).The Hedgehog signaling network.AMERICAN JOURNAL OF MEDICAL GENETICS PART A(1),5-28.
MLA Cohen, MM."The Hedgehog signaling network".AMERICAN JOURNAL OF MEDICAL GENETICS PART A .1(2003):5-28.
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