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For more information about Ollier's disease / Enchondromatosis, please contact the following organization.
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Research authored by Dr. Alman
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List of Publications via PubMed
(NIH National Library of Medicine)
List of Publications via PubMed
(NIH National Library of Medicine)
P53 and Rb in Cartilage Tumours in Mice
Abstract 2005 MHE Conference
Aneta Stojanovski, Louisa Ho, and Benjamin Alman
The Program in Developmental Biology and Division of Orthopaedic Surgery, The Hospital
for Sick Children Toronto, Ontario Canada
Chondrosarcomas can arise from being cartilage lesions, such as enchondromas and
osteochondromas.
In enchondromatosis, there is a high rate of malignant change, reported to be as high as 50% in
cases of Mafucci syndrome. Cytogenetic and mutational analysis studies identified mutations or
deletions in p53 or Rb in roughly one third of chondrosarcomas. As such, we examined the role of
these tumor suppressor genes using a mouse model of enchondromatosis.
We crossed p53 and Rb knockout mice with mice overexpressing Gli2 driven by the type II collagen
regulatory elements. Mice were sacrificed and limbs analyzed using histology, Safranin-O staining,
type X collagen immunohistochemistry, proliferation rate and apoptosis rate. Larger, hypercellular,
cartilaginous lesions containing pleomorphic cells arose in the Gli2;p53+/-, at an increasing incidence
starting at 2 months of age. By 8 months, 75% of these mice developed these larger lesions. This
was associated with an increase in cell proliferation. Gli2;Rb+/- mice also developed these larger
lesions, but only at 8 months of age.
Examination of the fetal limbs showed an expanded growth plate, involving all zones in the
Gli2;p53-/- mice, compared to the other genotypes.P53 deficiency modulates the effect of
overexpression of Gli2 in chondrocytes, resulting in a change in the growth plate and the
development of larger, hypercellular cartilage lesions, perhaps by increasing the number of
chondrocyte cells in the growth plate. This data also suggests that tumor suppressor genes play a
role in cartilaginous neoplasia.
Abstract 2005 MHE Conference
Aneta Stojanovski, Louisa Ho, and Benjamin Alman
The Program in Developmental Biology and Division of Orthopaedic Surgery, The Hospital
for Sick Children Toronto, Ontario Canada
Chondrosarcomas can arise from being cartilage lesions, such as enchondromas and
osteochondromas.
In enchondromatosis, there is a high rate of malignant change, reported to be as high as 50% in
cases of Mafucci syndrome. Cytogenetic and mutational analysis studies identified mutations or
deletions in p53 or Rb in roughly one third of chondrosarcomas. As such, we examined the role of
these tumor suppressor genes using a mouse model of enchondromatosis.
We crossed p53 and Rb knockout mice with mice overexpressing Gli2 driven by the type II collagen
regulatory elements. Mice were sacrificed and limbs analyzed using histology, Safranin-O staining,
type X collagen immunohistochemistry, proliferation rate and apoptosis rate. Larger, hypercellular,
cartilaginous lesions containing pleomorphic cells arose in the Gli2;p53+/-, at an increasing incidence
starting at 2 months of age. By 8 months, 75% of these mice developed these larger lesions. This
was associated with an increase in cell proliferation. Gli2;Rb+/- mice also developed these larger
lesions, but only at 8 months of age.
Examination of the fetal limbs showed an expanded growth plate, involving all zones in the
Gli2;p53-/- mice, compared to the other genotypes.P53 deficiency modulates the effect of
overexpression of Gli2 in chondrocytes, resulting in a change in the growth plate and the
development of larger, hypercellular cartilage lesions, perhaps by increasing the number of
chondrocyte cells in the growth plate. This data also suggests that tumor suppressor genes play a
role in cartilaginous neoplasia.
Benjamin Alman, M.D.
| Dr. Alman's research |
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By the Health On the Net Foundation. Click here to verify.# HON Conduct 282463 and is linked on the NIH
National Library of Medicine, Directory of Health Organizations (SIS) website, as well as the link for Patient
Information on The Diseases Database a cross-referenced index of human disease, and the Intute: health & life
sciences a free online service providing access to the very best Web resources for education and research
located in the UK
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