PMID- 12024028
OWN - NLM
STAT- MEDLINE
DCOM- 20020624
LR  - 20210526
IS  - 0270-7306 (Print)
IS  - 1098-5549 (Electronic)
IS  - 0270-7306 (Linking)
VI  - 22
IP  - 12
DP  - 2002 Jun
TI  - Sim2 mutants have developmental defects not overlapping with those of Sim1 
      mutants.
PG  - 4147-57
AB  - The mouse genome contains two Sim genes, Sim1 and Sim2. They are presumed to be 
      important for central nervous system (CNS) development because they are 
      homologous to the Drosophila single-minded (sim) gene, mutations in which cause a 
      complete loss of CNS midline cells. In the mammalian CNS, Sim2 and Sim1 are 
      coexpressed in the paraventricular nucleus (PVN). While Sim1 is essential for the 
      development of the PVN (J. L. Michaud, T. Rosenquist, N. R. May, and C.-M. Fan, 
      Genes Dev. 12:3264-3275, 1998), we report here that Sim2 mutant has a normal PVN. 
      Analyses of the Sim1 and Sim2 compound mutants did not reveal obvious genetic 
      interaction between them in PVN histogenesis. However, Sim2 mutant mice die 
      within 3 days of birth due to lung atelectasis and breathing failure. We 
      attribute the diminished efficacy of lung inflation to the compromised structural 
      components surrounding the pleural cavity, which include rib protrusions, 
      abnormal intercostal muscle attachments, diaphragm hypoplasia, and pleural 
      mesothelium tearing. Although each of these structures is minimally affected, we 
      propose that their combined effects lead to the mechanical failure of lung 
      inflation and death. Sim2 mutants also develop congenital scoliosis, reflected by 
      the unequal sizes of the left and right vertebrae and ribs. The temporal and 
      spatial expression patterns of Sim2 in these skeletal elements suggest that Sim2 
      regulates their growth and/or integrity.
FAU - Goshu, Eleni
AU  - Goshu E
AD  - Department of Embryology, Carnegie Institution of Washington, Baltimore, Maryland 
      21210, USA.
FAU - Jin, Hui
AU  - Jin H
FAU - Fasnacht, Rachel
AU  - Fasnacht R
FAU - Sepenski, Mike
AU  - Sepenski M
FAU - Michaud, Jacques L
AU  - Michaud JL
FAU - Fan, Chen-Ming
AU  - Fan CM
LA  - eng
GR  - R01 HD035596/HD/NICHD NIH HHS/United States
GR  - R01 HD35596/HD/NICHD NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Mol Cell Biol
JT  - Molecular and cellular biology
JID - 8109087
RN  - 0 (Basic Helix-Loop-Helix Transcription Factors)
RN  - 0 (Repressor Proteins)
RN  - 0 (SIM1 protein, human)
RN  - 0 (Sim1 protein, mouse)
RN  - 0 (Sim2 protein, mouse)
RN  - 0 (Transcription Factors)
SB  - IM
MH  - Animals
MH  - Animals, Newborn
MH  - Basic Helix-Loop-Helix Transcription Factors
MH  - Central Nervous System/growth & development/metabolism
MH  - Diaphragm/abnormalities
MH  - Face/embryology
MH  - Female
MH  - *Gene Expression Regulation, Developmental
MH  - Lung/pathology
MH  - Male
MH  - Mesoderm
MH  - Mice
MH  - Mice, Mutant Strains
MH  - Obesity/genetics
MH  - Paraventricular Hypothalamic Nucleus/growth & development
MH  - Pulmonary Atelectasis/genetics/physiopathology
MH  - Repressor Proteins/*genetics/metabolism
MH  - Respiration/*genetics
MH  - Ribs/abnormalities
MH  - Spine/abnormalities
MH  - Transcription Factors/*genetics/metabolism
PMC - PMC133848
EDAT- 2002/05/25 10:00
MHDA- 2002/06/25 10:01
PMCR- 2002/06/01
CRDT- 2002/05/25 10:00
PHST- 2002/05/25 10:00 [pubmed]
PHST- 2002/06/25 10:01 [medline]
PHST- 2002/05/25 10:00 [entrez]
PHST- 2002/06/01 00:00 [pmc-release]
AID - 0104 [pii]
AID - 10.1128/MCB.22.12.4147-4157.2002 [doi]
PST - ppublish
SO  - Mol Cell Biol. 2002 Jun;22(12):4147-57. doi: 10.1128/MCB.22.12.4147-4157.2002.