IKBKG/NEMO

Publications

Scheuerle A E et al. Incontinentia Pigmenti. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. 1999.  PMID: 20301645.

Boisson B et al. Rescue of recurrent deep intronic mutation underlying cell type-dependent quantitative NEMO deficiency. J Clin Invest. 2018.  PMID: 30422821.

Fusco F et al. Unusual Father-to-Daughter Transmission of Incontinentia Pigmenti Due to Mosaicism in IP Males. Pediatrics. 2017;140(3).  PMID: 28794079.

Fusco F et al. EDA-ID and IP, two faces of the same coin: how the same IKBKG/NEMO mutation affecting the NF-κB pathway can cause immunodeficiency and/or inflammation. Int Rev Immunol. 2015;34(6):445-59.  PMID: 26269396.

Fusco F et al. Incontinentia pigmenti: report on data from 2000 to 2013. Orphanet J Rare Dis. 2014;9:93.  PMID: 24961275.

Pizzamiglio M R et al. Incontinentia pigmenti: learning disabilities are a fundamental hallmark of the disease. PLoS One. 2014;9(1):e87771.  PMID: 24489960.

Conte MI et al. Insight into IKBKG/NEMO locus: report of new mutations and complex genomic rearrangements leading to incontinentia pigmenti disease. Hum Mutat. 2014;35(2):165-77.  PMID: 24339369.

Fusco F et al. Genomic architecture at the Incontinentia Pigmenti locus favours de novo pathological alleles through different mechanisms. Hum Mol  Genet. 2012;21(6):1260-71.  PMID: 22121116.

Fusco F et al. Alterations of the IKBKG locus and diseases: an update and a report of 13 novel mutations. Hum Mutat. 2008;29(5):595-604.  PMID: 18350553.

Fusco F et al. Microdeletion/duplication at the Xq28 IP locus causes a de novo IKBKG/NEMO/IKKgamma exon4_10 deletion in families with Incontinentia Pigmenti. Hum Mutat. 2009;30(9):1284-91.  PMID: 19603533.

Sebban-Benin H et al. Identification of TRAF6-dependent NEMO polyubiquitination sites through analysis of a new NEMO mutation causing incontinentia pigmenti. Hum Mol Genet. 2007;16(23):2805-15.  PMID: 17728323.

Fusco F et al. Clinical diagnosis of incontinentia pigmenti in a cohort of male patients. J Am Acad Dermatol. 2007;56(2):264-7.  PMID: 17224368.

Fusco F et al. The LCR at the IKBKG locus is prone to recombine. Am J Hum Genet. 2010;86(4):650-2; author reply 652-3.  PMID: 20380930.

Fusco F et al. Molecular analysis of the genetic defect in a large cohort of IP patients and identification of novel NEMO mutations interfering with NF-kappaB activation. Hum Mol Genet. 2004;13(16):1763-73.  PMID: 15229184.

Bardaro T et al. Two cases of misinterpretation of molecular results in incontinentia pigmenti, and a PCR-based method to discriminate NEMO/IKKgamma gene deletion. Hum Mutat. 2003;21(1):8-11.  PMID: 12497627.

Kenwrick S et al. Survival of male patients with incontinentia pigmenti carrying a lethal mutation can be explained by somatic mosaicism or Klinefelter syndrome. Am J Hum Genet. 2001;69(6):1210-7.  PMID: 11673821.

Döffinger R et al. X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-kappaB signaling. Nat Genet. 2001;27(3):277-85.  PMID:11242109.

Smahi A et al. Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti. Nature. 2000;405(6785):466-72.  PMID: 10839543.