Searching across hundreds of databases

Our searching services are busy right now. Your search will reload in five seconds.

Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

NEMO/IKK gamma-deficient mice model incontinentia pigmenti.

Molecular cell | Jun 15, 2000

Disruption of the X-linked gene encoding NF-kappa B essential modulator (NEMO) produces male embryonic lethality, completely blocks NF-kappa B activation by proinflammatory cytokines, and interferes with the generation and/or persistence of lymphocytes. Heterozygous female mice develop patchy skin lesions with massive granulocyte infiltration and hyperproliferation and increased apoptosis of keratinocytes. Diseased animals present severe growth retardation and early mortality. Surviving mice recover almost completely, presumably through clearing the skin of NEMO-deficient keratinocytes. Male lethality and strikingly similar skin lesions in heterozygous females are hallmarks of the human genetic disorder incontinentia pigmenti (IP). Together with the recent discovery that mutations in the human NEMO gene cause IP, our results indicate that we have created a mouse model for that disease.

Pubmed ID: 10911992 RIS Download

Mesh terms: Animals | Apoptosis | Cell Division | Cells, Cultured | Chemotaxis, Leukocyte | Cytokines | Disease Models, Animal | Female | Gene Targeting | Heterozygote | Humans | Hyperpigmentation | I-kappa B Kinase | Incontinentia Pigmenti | Keratinocytes | Liver | Male | Melanins | Mice | Mice, Knockout | NF-kappa B | Nitric Oxide Synthase | Nitric Oxide Synthase Type II | Protein-Serine-Threonine Kinases | Skin | Up-Regulation

Research resources used in this publication

None found

Research tools detected in this publication

None found

Data used in this publication

None found

Associated grants


Mouse Genome Informatics (Data, Gene Annotation)

Publication data is provided by the National Library of Medicine ® and PubMed ®. Data is retrieved from PubMed ® on a weekly schedule. For terms and conditions see the National Library of Medicine Terms and Conditions.

We have not found any resources mentioned in this publication.