Late-onset X-linked adrenal hypoplasia (DAX-1, NR0B1): two new adult-onset cases from a single center

被引:0
作者
Nikolaos Kyriakakis
Tolulope Shonibare
Julie Kyaw-Tun
Julie Lynch
Carlos F. Lagos
John C. Achermann
Robert D. Murray
机构
[1] St James’s University Hospital,Department of Endocrinology, Leeds Centre for Diabetes & Endocrinology
[2] Leeds Teaching Hospitals NHS Trust,Leeds Institute of Cardiovascular and Metabolic Medicine
[3] University of Leeds,Department of Endocrinology, School of Medicine
[4] Pontificia Universidad Católica de Chile,Facultad de Ciencia
[5] Universidad San Sebastián,Genetics & Genomic Medicine
[6] UCL Great Ormond Street Institute of Child Health,undefined
来源
Pituitary | 2017年 / 20卷
关键词
DAX-1; Adrenal hypoplasia congenita; Adrenal insufficiency; Hypogonadotropic hypogonadism; Repression helix domain; Nuclear receptors;
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中图分类号
学科分类号
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页码:585 / 593
页数:8
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[1]  
Muscatelli F(1994)Mutations in the DAX-1 gene give rise to both X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism Nature 372 672-676
[2]  
Strom TM(1994)An unusual member of the nuclear hormone receptor superfamily responsible for X-linked adrenal hypoplasia congenita Nature 372 635-641
[3]  
Walker AP(1999)Clinical and functional effects of mutations in the DAX-1 gene in patients with adrenal hypoplasia congenita J Clin Endocrinol Metab 84 504-511
[4]  
Zanaria E(2010)Clinical and genetic heterogeneity of congenital adrenal hypoplasia due to NR0B1 gene mutations Clin Endocrinol 72 448-454
[5]  
Récan D(2015)DAX-1 (NR0B1) and steroidogenic factor-1 (SF-1, NR5A1) in human disease Best Pract Res Clin Endocrinol Metab 29 607-619
[6]  
Meindl A(2006)Analysis of DAX1 (NR0B1) and steroidogenic factor-1 (NR5A1) in children and adults with primary adrenal failure: ten years’ experience J Clin Endocrinol Metab 91 3048-3054
[7]  
Bardoni B(1993)Comparative protein modelling by satisfaction of spatial restraints J Mol Biol 234 779-815
[8]  
Guioli S(2008)The structure of corepressor Dax-1 bound to its target nuclear receptor LRH-1 Proc Natl Acad Sci USA 105 18390-18395
[9]  
Zehetner G(2009)CHARMM: the biomolecular simulation program J Comput Chem 30 1545-1614
[10]  
Rabl W(1997)DAX-1 inhibits SF-1-mediated transactivation via a carboxy-terminal domain that is deleted in adrenal hypoplasia congenita Mol Cell Biol 17 1476-1483