PMID- 18204792
OWN - NLM
STAT- MEDLINE
DCOM- 20080307
LR  - 20211203
IS  - 1107-3756 (Print)
IS  - 1107-3756 (Linking)
VI  - 21
IP  - 2
DP  - 2008 Feb
TI  - Tuberin-heterozygous cell line TSC2ang1 as a model for tuberous 
      sclerosis-associated skin lesions.
PG  - 245-50
AB  - Tuberous sclerosis (TS), neurological disorder manifesting with the formation of 
      tumors in numerous organ systems, is a disease associated with the upregulation 
      of mammalian target of rapamycin (mTOR) pathway. It has been found that in 
      healthy individuals two tumor suppressor genes, TSC1 and TSC2, encoding proteins 
      called hamartin and tuberin, respectively, are responsible for the control over 
      mTOR kinase. Loss of one of these genes constitutes the genetic background of TS. 
      In the current study, we aimed at evaluating the fitness of the only 
      TS-associated sarcoma cell line deposited in American Tissue Culture Collection, 
      TSC2ang1, for the in vitro studies on TS. We found that the line shows a stable 
      chromosome pattern with typical Robertsonian translocations. Similarly to primary 
      tumors from TS patients, TSC2ang1 cells respond to rapamycin-induced mTOR 
      inhibition. The cells demonstrate activation of both Akt and Erk pathways, but 
      inhibition of neither of them is as effective as mTOR suppression when 
      considering proliferation potential. Based on these results we propose TSC2ang1 
      as a good and stable model for pathophysiological and pharmacological studies on 
      skin lesions in TS.
FAU - Wlodarski, Pawel K
AU  - Wlodarski PK
AD  - Department of Histology and Embryology, Center for Biostructure Research, Medical 
      University of Warsaw, Poland.
FAU - Maksym, Radoslaw
AU  - Maksym R
FAU - Oldak, Monika
AU  - Oldak M
FAU - Jozwiak, Sergiusz
AU  - Jozwiak S
FAU - Wojcik, Andrzej
AU  - Wojcik A
FAU - Jozwiak, Jaroslaw
AU  - Jozwiak J
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Greece
TA  - Int J Mol Med
JT  - International journal of molecular medicine
JID - 9810955
RN  - 0 (Androstadienes)
RN  - 0 (Butadienes)
RN  - 0 (Chromones)
RN  - 0 (Morpholines)
RN  - 0 (Nitriles)
RN  - 0 (Phosphoinositide-3 Kinase Inhibitors)
RN  - 0 (TSC2 protein, human)
RN  - 0 (Tuberous Sclerosis Complex 2 Protein)
RN  - 0 (Tumor Suppressor Proteins)
RN  - 0 (U 0126)
RN  - 31M2U1DVID (2-(4-morpholinyl)-8-phenyl-4H-1-benzopyran-4-one)
RN  - EC 2.7.- (Protein Kinases)
RN  - EC 2.7.1.1 (MTOR protein, human)
RN  - EC 2.7.11.1 (Proto-Oncogene Proteins c-akt)
RN  - EC 2.7.11.1 (TOR Serine-Threonine Kinases)
RN  - EC 2.7.11.24 (Extracellular Signal-Regulated MAP Kinases)
RN  - XVA4O219QW (Wortmannin)
SB  - IM
MH  - Androstadienes/pharmacology
MH  - Animals
MH  - Butadienes/pharmacology
MH  - CHO Cells
MH  - Cell Line
MH  - Cell Proliferation/drug effects
MH  - Chromones/pharmacology
MH  - Chromosomes, Human
MH  - Cricetinae
MH  - Cricetulus
MH  - Enzyme Activation/drug effects
MH  - Extracellular Signal-Regulated MAP Kinases/antagonists & inhibitors
MH  - Heterozygote
MH  - Humans
MH  - Karyotyping
MH  - Morpholines/pharmacology
MH  - Nitriles/pharmacology
MH  - Phosphoinositide-3 Kinase Inhibitors
MH  - Protein Kinases/metabolism
MH  - Proto-Oncogene Proteins c-akt/metabolism
MH  - Skin Neoplasms/enzymology/*pathology
MH  - Substrate Specificity/drug effects
MH  - TOR Serine-Threonine Kinases
MH  - Tuberous Sclerosis/enzymology/*pathology
MH  - Tuberous Sclerosis Complex 2 Protein
MH  - Tumor Suppressor Proteins/*genetics
MH  - Wortmannin
EDAT- 2008/01/22 09:00
MHDA- 2008/03/08 09:00
CRDT- 2008/01/22 09:00
PHST- 2008/01/22 09:00 [pubmed]
PHST- 2008/03/08 09:00 [medline]
PHST- 2008/01/22 09:00 [entrez]
PST - ppublish
SO  - Int J Mol Med. 2008 Feb;21(2):245-50.