{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T12:57:59Z","timestamp":1791291479222,"version":"4.3.0"},"reference-count":27,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"58","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Sci. STKE"],"published-print":{"date-parts":[[2000,11,14]]},"abstract":"<jats:p>The components of the nuclear factor-\u03baB (NF-\u03baB) family of transcription factors are critical for regulating the response to immune challenges. Recently, a role for NF-\u03baB in skin biology has been revealed. Within the cascade of proteins whose activities impinge upon the activation of NF-\u03baB, the NEMO (NF-\u03baB essential modulator)\/IKK\u03b3 protein is required for the activation of the I\u03baB kinases, which in turn, promote the degradation of I\u03baB proteins, leading to the derepression of NF-\u03baB activity. Courtois and Israe\u0308l discuss the role of NEMO\/IKK\u03b3 in normal physiological activation of NF-\u03baB and the consequences of defective NF-\u03baB activation, as an effect of NEMO\/IKK\u03b3 mutations, which can lead to incontinentia pigmenti, a disease marked by alopecia, tooth eruption, skin lesions, and changes in skin pigmentation.<\/jats:p>","DOI":"10.1126\/stke.2000.58.pe1","type":"journal-article","created":{"date-parts":[[2003,5,10]],"date-time":"2003-05-10T00:47:47Z","timestamp":1052527667000},"source":"Crossref","is-referenced-by-count":10,"title":["NF-\u03baB Defects in Humans: The NEMO\/Incontinentia Pigmenti Connection"],"prefix":"10.1126","volume":"2000","author":[{"given":"Gilles","family":"Courtois","sequence":"first","affiliation":[{"name":"The authors are in the Unite\u0301 de Biologie Mole\u0301culaire de l'Expression Ge\u0301nique, URA 1773 CNRS, Institut Pasteur 75724 Paris Cedex 15, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Israe\u0308l","sequence":"additional","affiliation":[{"name":"The authors are in the Unite\u0301 de Biologie Mole\u0301culaire de l'Expression Ge\u0301nique, URA 1773 CNRS, Institut Pasteur 75724 Paris Cedex 15, France."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_3_1_1_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.16.1.225"},{"key":"e_1_3_1_2_2","doi-asserted-by":"crossref","unstructured":"Rothwarf D.M. and Karin M. (1999) The NF-\u03baB activation pathway: A paradigm in information transfer from membrane to nucleus. Science's STKE. http:\/\/www.stke.org\/cgi\/content\/full\/OC_sigtrans;1999\/5\/re1.","DOI":"10.1126\/scisignal.51999re1"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.18.1.621"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0962-8924(00)01729-3"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81466-X"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1203236"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/376167a0"},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.6.2994"},{"key":"e_1_3_1_9_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.284.5412.321"},{"key":"e_1_3_1_10_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.189.11.1839"},{"key":"e_1_3_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)80042-4"},{"key":"e_1_3_1_12_2","doi-asserted-by":"crossref","first-page":"1729","DOI":"10.1101\/gad.14.14.1729","volume":"14","year":"2000","unstructured":"Li, Q., Estepa, G., M\u00e9met, S., Isra\u00ebl, A., and Verma, I.M. (2000) Complete lack of NF-\u03baB activity in IKK1 and IKK2 double-deficient mice: Additional defect in neurulation. Genes Dev. 14: 1729-1733.","journal-title":"Genes Dev."},{"key":"e_1_3_1_13_2","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1101\/gad.14.7.854","volume":"14","year":"2000","unstructured":"Rudolph, D., Yeh, W.C., Wakeham, A., Rudolph, B., Nallainathan, D., Elia, A., Potter, J., and Mak, T.W. (2000) Severe liver degeneration and lack of NF-\u03baB activation in NEMO\/IKK\u03b3 deficient mice. Genes Dev. 14: 854-862.","journal-title":"Genes Dev."},{"key":"e_1_3_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(00)80262-2"},{"key":"e_1_3_1_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(00)80263-4"},{"key":"e_1_3_1_16_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.149.5.999"},{"key":"e_1_3_1_17_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.5.2307"},{"key":"e_1_3_1_18_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI7630"},{"key":"e_1_3_1_19_2","first-page":"3299","volume":"59","year":"1999","unstructured":"van Hogerlinden, M., Rozell, B.L., Ahrlund-Richter, L., and Toftgard, R. (1999) Squamous cell carcinomas and increased apoptosis in skin with inhibited Rel\/nuclear factor-\u03baB signaling. Cancer Res. 59: 3299-3303.","journal-title":"Cancer Res."},{"key":"e_1_3_1_20_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.284.5412.316"},{"key":"e_1_3_1_21_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.13.10.1322"},{"key":"e_1_3_1_22_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.284.5412.313"},{"key":"e_1_3_1_23_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.53.37957"},{"key":"e_1_3_1_24_2","unstructured":"Mendelian inheritance MIM*308310; see also the National Incontinentia Pigmenti Foundation at http:\/\/imgen.bcm.tmc.edu\/NIPF\/."},{"key":"e_1_3_1_25_2","doi-asserted-by":"publisher","DOI":"10.1038\/35013114"},{"key":"e_1_3_1_26_2","first-page":"115","volume":"6","year":"1999","unstructured":"Jin, D.Y., and Jeang, K.T. (1999) Isolation of full-length cDNA and chromosomal localization of human NF-\u03baB modulator NEMO to Xq28. J. Biomed. Sci. 6: 115-120.","journal-title":"J. Biomed. Sci."},{"key":"e_1_3_1_27_2","doi-asserted-by":"crossref","first-page":"5994","DOI":"10.4049\/jimmunol.163.11.5994","volume":"163","year":"1999","unstructured":"M\u00e9met, S., Laouini, D., Epinat, J.C., Whiteside, S.T., Goudeau, B., Philpott, D., Kayal, S., Sansonetti, P.J., Berche, P., Kanellopoulos, J., and Isra\u00ebl, A. (1999) I\u03baB\u03f5-deficient mice: Reduction of one T cell precursor subspecies and enhanced Ig isotype switching and cytokine synthesis. J. Immunol. 163: 5994-6005.","journal-title":"J. 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