PMID- 10572046
OWN - NLM
STAT- MEDLINE
DCOM- 20000121
LR  - 20170922
IS  - 0950-1991 (Print)
IS  - 0950-1991 (Linking)
VI  - 126
IP  - 24
DP  - 1999 Dec
TI  - The murine Bapx1 homeobox gene plays a critical role in embryonic development of 
      the axial skeleton and spleen.
PG  - 5699-711
AB  - Our previous studies in both mouse and human identified the Bapx1 homeobox gene, 
      a member of the NK gene family, as one of the earliest markers for
      prechondrogenic cells that will subsequently undergo mesenchymal condensation,
      cartilage production and, finally, endochondral bone formation. In addition,
      Bapx1 is an early developmental marker for splanchnic mesoderm, consistent with a
      role in visceral mesoderm specification, a function performed by its homologue
      bagpipe, in Drosophila. The human homologue of Bapx1 has been identified and
      mapped to 4p16.1, a region containing loci for several skeletal diseases. Bapx1
      null mice are affected by a perinatal lethal skeletal dysplasia and asplenia,
      with severe malformation or absence of specific bones of the vertebral column and
      cranial bones of mesodermal origin, with the most severely affected skeletal
      elements corresponding to ventral structures associated with the notochord. We
      provide evidence that the failure of the formation of skeletal elements in Bapx1 
      null embryos is a consequence of a failure of cartilage development, as
      demonstrated by downregulation of several molecular markers required for normal
      chondroblast differentiation (&agr; 1(II) collagen, Fgfr3, Osf2, Indian hedgehog,
      Sox9), as well as a chondrocyte-specific alpha1 (II) collagen-lacZ transgene. The
      cartilage defects are correlated with failed differentiation of the sclerotome at
      the time when these cells are normally initiating chondrogenesis. Loss of Bapx1
      is accompanied by an increase in apoptotic cell death in affected tissues,
      although cell cycling rates are unaltered.
FAU - Tribioli, C
AU  - Tribioli C
AD  - Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of
      Medicine, One Gustave L. Levy Place, New York, NY 10029-6574, USA.
FAU - Lufkin, T
AU  - Lufkin T
LA  - eng
GR  - D.075/Telethon/Italy
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Development
JT  - Development (Cambridge, England)
JID - 8701744
RN  - 0 (BMP4 protein, human)
RN  - 0 (Bmp4 protein, mouse)
RN  - 0 (Bone Morphogenetic Protein 4)
RN  - 0 (Bone Morphogenetic Proteins)
RN  - 0 (Core Binding Factor Alpha 1 Subunit)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (Hedgehog Proteins)
RN  - 0 (Homeodomain Proteins)
RN  - 0 (NKX3-2 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Nkx3-2 protein, mouse)
RN  - 0 (Paired Box Transcription Factors)
RN  - 0 (Proteins)
RN  - 0 (Receptors, Fibroblast Growth Factor)
RN  - 0 (Trans-Activators)
RN  - 0 (Transcription Factors)
RN  - 142661-96-9 (PAX1 transcription factor)
RN  - 9007-34-5 (Collagen)
RN  - EC 2.7.10.1 (FGFR3 protein, human)
RN  - EC 2.7.10.1 (Fgfr3 protein, mouse)
RN  - EC 2.7.10.1 (Protein-Tyrosine Kinases)
RN  - EC 2.7.10.1 (Receptor, Fibroblast Growth Factor, Type 3)
SB  - IM
MH  - Animals
MH  - *Bone Development
MH  - Bone Morphogenetic Protein 4
MH  - Bone Morphogenetic Proteins/genetics
MH  - Cartilage, Articular/embryology
MH  - Collagen/genetics
MH  - Core Binding Factor Alpha 1 Subunit
MH  - DNA-Binding Proteins/genetics
MH  - Female
MH  - Gene Expression Regulation, Developmental
MH  - Gene Targeting
MH  - Genes, Homeobox
MH  - Hedgehog Proteins
MH  - Homeodomain Proteins/genetics/*physiology
MH  - Humans
MH  - Male
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Transgenic
MH  - *Neoplasm Proteins
MH  - Paired Box Transcription Factors
MH  - *Protein-Tyrosine Kinases
MH  - Proteins/genetics
MH  - Receptor, Fibroblast Growth Factor, Type 3
MH  - Receptors, Fibroblast Growth Factor/genetics
MH  - Spine/*embryology
MH  - Spleen/*embryology
MH  - *Trans-Activators
MH  - Transcription Factors/genetics
EDAT- 1999/11/26 00:00
MHDA- 1999/11/26 00:01
CRDT- 1999/11/26 00:00
PHST- 1999/11/26 00:00 [pubmed]
PHST- 1999/11/26 00:01 [medline]
PHST- 1999/11/26 00:00 [entrez]
PST - ppublish
SO  - Development. 1999 Dec;126(24):5699-711.