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Title: Delineation of a contiguous gene syndrome with multiple exostoses, enlarged parietal foramina, craniofacial dysostosis, and mental retardation, caused by deletions on the short arm of chromosome 11

Journal Article · · American Journal of Human Genetics
OSTI ID:285054
; ;  [1]; ;  [2]
  1. Univ. Hospital Dresden (Germany)
  2. Univ. of Antwerp (Belgium); and others

A contiguous gene syndrome due to deletions of the proximal short arm of chromosome 11 is described in eight patients belonging to four families. The main clinical features are multiple exostoses, enlarged parietal foramina, craniofacial dysostosis, and mental retardation. The patients have cytogenetic and/or molecular deletions of chromosome 11p11-p13. These deletions are located between the centromere and D11S914 in a region of {approximately}20 cM. The present study confirms the presence of a multiple exostoses gene on chromosome 11p. Furthermore, it suggests that the gene for isolated foramina parietalia permagna and genes associated with craniofacial dysostosis and mental retardation reside in the same chromosomal region. 31 refs., 5 figs., 1 tab.

OSTI ID:
285054
Journal Information:
American Journal of Human Genetics, Vol. 58, Issue 4; Other Information: PBD: Apr 1996
Country of Publication:
United States
Language:
English