PMID- 16933211
OWN - NLM
STAT- MEDLINE
DCOM- 20061222
LR  - 20060830
IS  - 1542-0752 (Print)
IS  - 1542-0752 (Linking)
VI  - 76
IP  - 6
DP  - 2006 Jun
TI  - Asymmetry of skeletal effects of Dominant hemimelia.
PG  - 474-82
AB  - BACKGROUND: Dominant hemimelia (Dh) is a dominant mutation that arose 
      spontaneously in mice; Dh animals exhibit reduced numbers of lumbar vertebrae and 
      preaxial hindlimb defects. Absence of spleen occurs in both Dh/+ and Dh/Dh 
      animals. This study was undertaken to characterize asymmetry of skeletal defects 
      in the Dh mouse, specifically hindlimb asymmetries in association with axial 
      defects. METHODS: A total of 29 Dh/+ and 100 +/+ fetuses (gestational day [GD] 
      18) were identified by phenotype and linked DNA and their skeletons were 
      analyzed. RESULTS: The results revealed an asymmetry of hindlimb skeletal defects 
      in Dh/+ animals. In +/+ fetuses, the left and right tibia were symmetrical with 
      99.0% of the animals possessing 6 lumbar vertebrae. However, Dh/+ fetuses showed 
      asymmetry in length of left and right tibia and a reduction to 5 lumbar vertebrae 
      in 86.2% of animals. There was a range from mild to severe asymmetry as evidenced 
      by direct comparison of the length of the left to the right tibia of each animal. 
      Tibial shortening was greater on the left than the right in 65.5% of Dh/+ 
      fetuses; only 20.7% had symmetrical tibia. Oligodactyly, defined as absence of 
      the first or second toe, was similarly more frequent on the left. CONCLUSIONS: 
      Asymmetry is characteristic of many human limb malformations, although analysis 
      of the molecular basis is difficult. Therefore, Dh/+ mice, which exhibit reduced 
      numbers of lumbar vertebrae, asymmetric hindlimb defects, and complete absence of 
      spleen, provide an important model for studying the relationship between axial 
      patterning and asymmetric skeletal defects.
CI  - (c) 2006 Wiley-Liss, Inc.
FAU - Owen, Mary H
AU  - Owen MH
AD  - Simmons College, Biology Department, Boston, Massachusetts 02115-5898, USA. 
      mary.owen@simmons.edu
FAU - Coull, Brent A
AU  - Coull BA
FAU - Holmes, Lewis B
AU  - Holmes LB
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Birth Defects Res A Clin Mol Teratol
JT  - Birth defects research. Part A, Clinical and molecular teratology
JID - 101155107
SB  - IM
MH  - Animals
MH  - Ectromelia/genetics/*pathology
MH  - Female
MH  - *Genes, Dominant
MH  - Male
MH  - Mice
MH  - Mice, Inbred AKR
MH  - Mice, Inbred C3H
MH  - Mice, Inbred C57BL
MH  - Phenotype
MH  - Reproducibility of Results
EDAT- 2006/08/26 09:00
MHDA- 2006/12/23 09:00
CRDT- 2006/08/26 09:00
PHST- 2006/08/26 09:00 [pubmed]
PHST- 2006/12/23 09:00 [medline]
PHST- 2006/08/26 09:00 [entrez]
AID - 10.1002/bdra.20256 [doi]
PST - ppublish
SO  - Birth Defects Res A Clin Mol Teratol. 2006 Jun;76(6):474-82. doi: 
      10.1002/bdra.20256.