IKK1-deficient mice exhibit abnormal development of skin and skeleton

Qiutang Li, Qingxian Lu, Jason Y. Hwang, Dirk Büscher, Kuo Fen Lee, Juan Carlos Izpisua-Belmonte, Inder M. Verma*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

421 Scopus citations


IKB kinases (IKKs) IKK1 and IKK2 are two putative IκBα kinases involved in NF-κB activation. To examine the in vivo functions of IKK1, we generated IKK1-deficient mice. The mutant mice are perinatally lethal and exhibit a wide range of developmental defects. Newborn mutant mice have shiny, taut, and sticky skin without whiskers. Histological analysis shows thicker epidermis, which is unable to differentiate. Limbs and tail are wrapped inside the skin and do not extend properly out of the body trunk. Skeleton staining reveals a cleft secondary palate, split sternebra 6, and deformed incisors. NF-κB activation mediated by TNFα and IL-1 is diminished in IKK1-deficient mouse embryonic fibroblast (MEF) cells. The IKK complex in the absence of IKK1 is capable of phosphorylating IKBα and IKBβ in vitro. Our results support a role for IKK1 in NF-κB activation and uncover its involvement in skin and skeleton development. We conclude further that the two related kinases IKK1 and IKK2 have distinct functions and can not be substituted for each other's functions.

Original languageEnglish (US)
Pages (from-to)1322-1328
Number of pages7
JournalGenes and Development
Issue number10
StatePublished - May 15 1999
Externally publishedYes


  • IKK1
  • Limb
  • Mice
  • NF-κB
  • Skeleton
  • Skin

ASJC Scopus subject areas

  • Genetics
  • Developmental Biology


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