| pubmed-article:10911991 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0086418 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0015780 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0012634 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0037274 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0019425 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0021171 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0241764 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C0011155 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C1416380 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C1533711 | lld:lifeskim |
| pubmed-article:10911991 | lifeskim:mentions | umls-concept:C2348205 | lld:lifeskim |
| pubmed-article:10911991 | pubmed:issue | 6 | lld:pubmed |
| pubmed-article:10911991 | pubmed:dateCreated | 2000-8-15 | lld:pubmed |
| pubmed-article:10911991 | pubmed:abstractText | IKK gamma/NEMO is the essential regulatory subunit of the I kappa B kinase (IKK), encoded by an X-linked gene in mice and humans. It is required for NF-kappa B activation and resistance to TNF-induced apoptosis. Female mice heterozygous for Ikk gamma/Nemo deficiency develop a unique dermatopathy characterized by keratinocyte hyperproliferation, skin inflammation, hyperkeratosis, and increased apoptosis. Although Ikk gamma+/- females eventually recover, Ikk gamma- males die in utero. These symptoms and inheritance pattern are very similar to those of incontinentia pigmenti (IP), a human genodermatosis, synthenic with the IKK gamma/NEMO locus. Indeed, biopsies and cells from IP patients exhibit defective IKK gamma/NEMO expression but normal expression of IKK catalytic subunits. This unique self-limiting disease, the first to be genetically linked to the IKK signaling pathway, is dependent on X-chromosome inactivation. We propose that the IKK gamma/NEMO-deficient cells trigger an inflammatory reaction that eventually leads to their death. | lld:pubmed |
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| pubmed-article:10911991 | pubmed:language | eng | lld:pubmed |
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| pubmed-article:10911991 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:10911991 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:10911991 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:10911991 | pubmed:month | Jun | lld:pubmed |
| pubmed-article:10911991 | pubmed:issn | 1097-2765 | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:TakahashiTT | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:RobertsJ LJL | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:SchwarzTT | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:JohnsonR SRS | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:FengLL | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:KarinMM | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:GodfreyV LVL | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:MakrisCC | lld:pubmed |
| pubmed-article:10911991 | pubmed:author | pubmed-author:Krähn-Senftle... | lld:pubmed |
| pubmed-article:10911991 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:10911991 | pubmed:volume | 5 | lld:pubmed |
| pubmed-article:10911991 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:10911991 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:10911991 | pubmed:pagination | 969-79 | lld:pubmed |
| pubmed-article:10911991 | pubmed:dateRevised | 2009-11-19 | lld:pubmed |
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| pubmed-article:10911991 | pubmed:year | 2000 | lld:pubmed |
| pubmed-article:10911991 | pubmed:articleTitle | Female mice heterozygous for IKK gamma/NEMO deficiencies develop a dermatopathy similar to the human X-linked disorder incontinentia pigmenti. | lld:pubmed |
| pubmed-article:10911991 | pubmed:affiliation | Department of Pharmacology, University of California, San Diego, La Jolla 92093, USA. | lld:pubmed |
| pubmed-article:10911991 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:10911991 | pubmed:publicationType | Research Support, U.S. Gov't, P.H.S. | lld:pubmed |
| pubmed-article:10911991 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:16151 | entrezgene:pubmed | pubmed-article:10911991 | lld:entrezgene |
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