Fetal inhibition of inflammation improves disease phenotypes in harlequin ichthyosis.

التفاصيل البيبلوغرافية
العنوان:	Fetal inhibition of inflammation improves disease phenotypes in harlequin ichthyosis.
المؤلفون:	Cottle DL; Department of Biochemistry and Molecular Biology., Ursino GM; Department of Biochemistry and Molecular Biology., Ip SC; Department of Biochemistry and Molecular Biology., Jones LK; Department of Biochemistry and Molecular Biology., Ditommaso T; Department of Biochemistry and Molecular Biology., Hacking DF; Department of Anaesthetics, Saint Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia Department of Paediatric Intensive Care, The Royal Children's Hospital, Melbourne, VIC, Australia., Mangan NE; Centre for Innate Immunity and Infectious Diseases., Mellett NA; Baker IDI Heart and Diabetes Institute, 75 Commercial Road, Melbourne, VIC 3004, Australia., Henley KJ; Walter and Eliza Hall Institute, 1G Royal Parade, Parkville, VIC 3052, Australia., Sviridov D; Baker IDI Heart and Diabetes Institute, 75 Commercial Road, Melbourne, VIC 3004, Australia., Nold-Petry CA; The Ritchie Centre, MIMR-PHI Institute of Medical Research, 27-31 Wright Street, Clayton, VIC 3168, Australia., Nold MF; The Ritchie Centre, MIMR-PHI Institute of Medical Research, 27-31 Wright Street, Clayton, VIC 3168, Australia., Meikle PJ; Baker IDI Heart and Diabetes Institute, 75 Commercial Road, Melbourne, VIC 3004, Australia., Kile BT; Walter and Eliza Hall Institute, 1G Royal Parade, Parkville, VIC 3052, Australia Department of Medical Biology, University of Melbourne, Parkville, VIC 3010, Australia and., Smyth IM; Department of Biochemistry and Molecular Biology Department of Anatomy and Developmental Biology, Monash University, Wellington Road, Clayton, VIC 3800, Australia ian.smyth@monash.edu.
المصدر:	Human molecular genetics [Hum Mol Genet] 2015 Jan 15; Vol. 24 (2), pp. 436-49. Date of Electronic Publication: 2014 Sep 10.
نوع المنشور:	Journal Article; Research Support, Non-U.S. Gov't
اللغة:	English
بيانات الدورية:	Publisher: IRL Press at Oxford University Press Country of Publication: England NLM ID: 9208958 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1460-2083 (Electronic) Linking ISSN: 09646906 NLM ISO Abbreviation: Hum Mol Genet Subsets: MEDLINE
أسماء مطبوعة:	Original Publication: Oxford, England ; New York : IRL Press at Oxford University Press, c1992-
مواضيع طبية MeSH:	Ichthyosis, Lamellar/embryology , Ichthyosis, Lamellar/immunology, Animals ; Cell Differentiation ; Chemokines/genetics ; Chemokines/immunology ; Disease Models, Animal ; Epidermis/embryology ; Epidermis/immunology ; Female ; Humans ; Ichthyosis, Lamellar/genetics ; Ichthyosis, Lamellar/physiopathology ; Interleukin-1/genetics ; Interleukin-1/immunology ; Keratinocytes/cytology ; Male ; Mice ; Mice, Knockout ; Phenotype ; Skin/embryology ; Skin/immunology
مستخلص:	Harlequin ichthyosis (HI) is a severe skin disease which leads to neonatal death in ∼50% of cases. It is the result of mutations in ABCA12, a protein that transports lipids required to establish the protective skin barrier needed after birth. To better understand the life-threatening newborn HI phenotype, we analysed the developing epidermis for consequences of lipid dysregulation in mouse models. We observed a pro-inflammatory signature which was characterized by chemokine upregulation in embryonic skin which is distinct from that seen in other types of ichthyosis. Inflammation also persisted in grafted HI skin. To examine the contribution of inflammation to disease development, we overexpressed interleukin-37b to globally suppress fetal inflammation, observing considerable improvements in keratinocyte differentiation. These studies highlight inflammation as an unexpected contributor to HI disease development in utero, and suggest that inhibiting inflammation may reduce disease severity. (© The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)
المشرفين على المادة:	0 (Chemokines) 0 (IL37 protein, human) 0 (Interleukin-1)
تواريخ الأحداث:	Date Created: 20140912 Date Completed: 20150923 Latest Revision: 20151218
رمز التحديث:	20221213
DOI:	10.1093/hmg/ddu459
PMID:	25209981
قاعدة البيانات:	MEDLINE

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تدمد:	1460-2083
DOI:	10.1093/hmg/ddu459