Knowledge Resource Center for Ecological Environment in Arid Area
DOI | 10.1002/ajmg.a.20495 |
The Hedgehog signaling network | |
Cohen, MM | |
通讯作者 | Cohen, MM |
会议名称 | Bryan D Hall Meeting 2003 |
会议日期 | MAR, 2003 |
会议地点 | LOS ANGELES, CALIFORNIA |
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 |
来源出版物 | AMERICAN JOURNAL OF MEDICAL GENETICS PART A |
ISSN | 0148-7299 |
出版年 | 2003 |
期号 | 1 |
页码 | 5-28 |
出版者 | WILEY-LISS |
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 | 会议论文 |
条目标识符 | http://119.78.100.177/qdio/handle/2XILL650/294068 |
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 |
推荐引用方式 GB/T 7714 | Cohen, MM. The Hedgehog signaling network[C]:WILEY-LISS,2003:5-28. |
条目包含的文件 | 条目无相关文件。 |
个性服务 |
推荐该条目 |
保存到收藏夹 |
导出为Endnote文件 |
谷歌学术 |
谷歌学术中相似的文章 |
[Cohen, MM]的文章 |
百度学术 |
百度学术中相似的文章 |
[Cohen, MM]的文章 |
必应学术 |
必应学术中相似的文章 |
[Cohen, MM]的文章 |
相关权益政策 |
暂无数据 |
收藏/分享 |
除非特别说明,本系统中所有内容都受版权保护,并保留所有权利。