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Severe liver degeneration and lack of NF-(kappa)B activation in NEMO/IKK(gamma)-deficient mice

Article Abstract:

Liver degeneration and lack of NF-(kappa)B activation have been studied in NEMO/IKK(gamma)-deficient mice. The mice were generated by gene targeting. Mutant embryos die early from severe liver damage from apoptosis. Fibroblasts lack detectable NR-(kappa)B DNA-binding activity as a response to TNG(alpha), Poly(IC), and IL-1 LPS. They do not have stimulus-dependent I(kappa)B kinase activity. Thus they show greater sensitivity to TNF(alpha)induced apoptosis. It appears that NEMO/IKK(gamma) is the first essential noncatalytic component of the IKK complex.

Author: Rudolph, Dorothea, Yeh, Wen-Chen, Wakeham, Andrew, Rudolph, Bettina, Nallainathan, Dhani, Potter, Julia, Elia, Andrew J., Mak, Tak W.
Publisher: Cold Spring Harbor Laboratory Press
Publication Name: Genes & Development
Subject: Biological sciences
ISSN: 0890-9369
Year: 2000
United Kingdom, Usage, Liver, Cell death, Cytochemistry, Mice, mutant strains, Mutant mice, Protein kinases, Fibroblasts

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The tumor suppressor gene Brca1 is required for embryonic cellular proliferation in the mouse

Article Abstract:

The tumor suppressor gene Brca1 appears to be a positive regulator of cellular growth in the early stages of embryogenesis in mice. Brca1 is necessary for the progression of the cell cycle in embryonic cells. Brca1(super +/-) mice are normal without any tumor-development until eleven months of age. However, homozygous Brca1(super 5-6) mutants die before 7.5 days of birth. Mutant embryos show no mesoderm formation and are improperly developed. Cell proliferation decreases without increased apoptosis, indicating the involvement of Brca1 in cell proliferation.

Author: Wakeham, Andrew, Potter, Julia, Mak, Tak W., Rossant, Janet, Hakem, Razqallah, Reitmair, Armin, Sirard, Christian, Hakem, Anne, Roberts, Jim, De la Pompa, Jose Luis, Mo, Rong, Woo, Minna, Billia, Filio, Firpo, Eduardo, Hui, Chi Chung
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1996
Mutation (Biology), Mutation, Tumor suppressor genes, Cell proliferation, Mice as laboratory animals, House mouse

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TRAF6 deficiency results in osteopetrosis and defective interleukin-1, CD40, and LPS signaling

Article Abstract:

Bone resorption and remodeling is a very precisely controlled body process that requires osteoclasts to function as a part of the process. Mice that lack the TNF receptor-associated factor 6 (TRAF6) are osteopetrotic with defects in tooth eruption and bone remodeling, the result of impaired osteoclast activity. TRAF6 has been shown to be crucial in IL-1 and CD40 signaling and also in lipopolysaccharide (LPS) signaling, which would not be expected. It is essential for survival perinatally and postnatally, also unexpected.

Author: Itie, Annick, Sarosi, Ildiko, Penninger, Josef, Yeh, Wen-Chen, Wakeham, Andrew, Mak, Tak W., Sasaki, Takehiko, Lacey, David L., Boyle, William J., Ho, Alexandra, Duncan, Gordon S., Goeddel, David V., Morony, Sean, Cao, Zhaodan, Khoo, Wilson, Dunstan, Colin R., Capparelli, Casey, Van, Gwyneth, Lomaga, Mark A., Furlonger, Caren, Kaufman, Stephen, Heiden, Annette van der, Paige, Christopher J.
Publisher: Cold Spring Harbor Laboratory Press
Publication Name: Genes & Development
Subject: Biological sciences
ISSN: 0890-9369
Year: 1999
United States, Canada, Genetic aspects, Developmental biology, Tumor necrosis factor, Interleukin-1, Osteopetrosis

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Subjects list: Research, Physiological aspects
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