PMID- 17015619
OWN - NLM
STAT- MEDLINE
DCOM- 20061128
LR  - 20211203
IS  - 0021-9525 (Print)
IS  - 1540-8140 (Electronic)
IS  - 0021-9525 (Linking)
VI  - 175
IP  - 1
DP  - 2006 Oct 9
TI  - Role of FIP200 in cardiac and liver development and its regulation of TNFalpha 
      and TSC-mTOR signaling pathways.
PG  - 121-33
AB  - Focal adhesion kinase family interacting protein of 200 kD (FIP200) has been 
      shown to regulate diverse cellular functions such as cell size, proliferation, 
      and migration in vitro. However, the function of FIP200 in vivo has not been 
      investigated. We show that targeted deletion of FIP200 in the mouse led to 
      embryonic death at mid/late gestation associated with heart failure and liver 
      degeneration. We found that FIP200 knockout (KO) embryos show reduced S6 kinase 
      activation and cell size as a result of increased tuberous sclerosis complex 
      function. Furthermore, FIP200 KO embryos exhibited significant apoptosis in heart 
      and liver. Consistent with this, FIP200 KO mouse embryo fibroblasts and liver 
      cells showed increased apoptosis and reduced c-Jun N-terminal kinase 
      phosphorylation in response to tumor necrosis factor (TNF) alpha stimulation, 
      which might be mediated by FIP200 interaction with apoptosis signal-regulating 
      kinase 1 (ASK1) and TNF receptor-associated factor 2 (TRAF2), regulation of 
      TRAF2-ASK1 interaction, and ASK1 phosphorylation. Together, our results reveal 
      that FIP200 functions as a regulatory node to couple two important signaling 
      pathways to regulate cell growth and survival during mouse embryogenesis.
FAU - Gan, Boyi
AU  - Gan B
AD  - Department of Molecular Medicine, College of Veterinary Medicine, Cornell 
      University, Ithaca, NY 14853, USA.
FAU - Peng, Xu
AU  - Peng X
FAU - Nagy, Tamas
AU  - Nagy T
FAU - Alcaraz, Ana
AU  - Alcaraz A
FAU - Gu, Hua
AU  - Gu H
FAU - Guan, Jun-Lin
AU  - Guan JL
LA  - eng
GR  - R01 GM052890/GM/NIGMS NIH HHS/United States
GR  - R01 HL073394/HL/NHLBI NIH HHS/United States
GR  - GM52890/GM/NIGMS NIH HHS/United States
GR  - HL73394/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
DEP - 20061002
PL  - United States
TA  - J Cell Biol
JT  - The Journal of cell biology
JID - 0375356
RN  - 0 (Autophagy-Related Proteins)
RN  - 0 (Intracellular Signaling Peptides and Proteins)
RN  - 0 (Rb1cc1 protein, mouse)
RN  - 0 (TNF Receptor-Associated Factor 2)
RN  - 0 (Tuberous Sclerosis Complex 1 Protein)
RN  - 0 (Tuberous Sclerosis Complex 2 Protein)
RN  - 0 (Tumor Necrosis Factor-alpha)
RN  - 0 (Tumor Suppressor Proteins)
RN  - EC 2.7.- (Protein Kinases)
RN  - EC 2.7.1.1 (mTOR protein, mouse)
RN  - EC 2.7.11.1 (Ribosomal Protein S6 Kinases)
RN  - EC 2.7.11.1 (TOR Serine-Threonine Kinases)
RN  - EC 2.7.11.24 (JNK Mitogen-Activated Protein Kinases)
RN  - EC 2.7.11.25 (MAP Kinase Kinase Kinase 5)
RN  - EC 2.7.11.25 (Map3k5 protein, mouse)
SB  - IM
MH  - Animals
MH  - Apoptosis
MH  - Autophagy-Related Proteins
MH  - Cell Size
MH  - Embryo, Mammalian/cytology/metabolism
MH  - Embryonic Development/physiology
MH  - Enzyme Activation
MH  - Fetal Death
MH  - Gene Deletion
MH  - Gestational Age
MH  - Heart/*embryology
MH  - Intracellular Signaling Peptides and Proteins/genetics/metabolism/*physiology
MH  - JNK Mitogen-Activated Protein Kinases/metabolism
MH  - Liver/cytology/*embryology/metabolism
MH  - MAP Kinase Kinase Kinase 5/metabolism
MH  - Mice
MH  - Myocardium/cytology/metabolism
MH  - Protein Kinases/*metabolism
MH  - Ribosomal Protein S6 Kinases/metabolism
MH  - *Signal Transduction
MH  - TNF Receptor-Associated Factor 2/metabolism
MH  - TOR Serine-Threonine Kinases
MH  - Tuberous Sclerosis Complex 1 Protein
MH  - Tuberous Sclerosis Complex 2 Protein
MH  - Tumor Necrosis Factor-alpha/*metabolism
MH  - Tumor Suppressor Proteins/*metabolism
PMC - PMC2064504
EDAT- 2006/10/04 09:00
MHDA- 2006/12/09 09:00
PMCR- 2007/04/09
CRDT- 2006/10/04 09:00
PHST- 2006/10/04 09:00 [pubmed]
PHST- 2006/12/09 09:00 [medline]
PHST- 2006/10/04 09:00 [entrez]
PHST- 2007/04/09 00:00 [pmc-release]
AID - jcb.200604129 [pii]
AID - 200604129 [pii]
AID - 10.1083/jcb.200604129 [doi]
PST - ppublish
SO  - J Cell Biol. 2006 Oct 9;175(1):121-33. doi: 10.1083/jcb.200604129. Epub 2006 Oct 
      2.