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Dan Wells, PH.D.
Dan Wells,
Professor Department of Biology and Biochemistry University of Houston
Multiple Hereditary Exostoses (MHE) is an autosomal dominant skeletal disorder most frequently
caused by mutations in the EXT1 gene.
MHE affects proper development of endochondral bones, such that all affected individuals present
with exostoses adjacent to the growth plate of long bones, while some individuals exhibit additional
bone deformities. EXT1 functions as a heparan sulfate (HS) co-polymerase, and when defective
causes improper elongation of glycosaminoglycan side chains on core proteins of HS proteoglycans.
Although analysis of heterozygous EXT1-deficient mice has failed to reveal any significant gross
morphological variations in skeletal development, significant alterations in molecular signaling occur in
the developing long bones.
Our results indicate that defects in EXT1 and the resulting reduction in HS lead to enhanced Indian
Hedgehog diffusion causing an increase in chondrocyte proliferation and delayed hypertrophic
differentiation.
Professor Department of Biology and Biochemistry University of Houston
Multiple Hereditary Exostoses (MHE) is an autosomal dominant skeletal disorder most frequently
caused by mutations in the EXT1 gene.
MHE affects proper development of endochondral bones, such that all affected individuals present
with exostoses adjacent to the growth plate of long bones, while some individuals exhibit additional
bone deformities. EXT1 functions as a heparan sulfate (HS) co-polymerase, and when defective
causes improper elongation of glycosaminoglycan side chains on core proteins of HS proteoglycans.
Although analysis of heterozygous EXT1-deficient mice has failed to reveal any significant gross
morphological variations in skeletal development, significant alterations in molecular signaling occur in
the developing long bones.
Our results indicate that defects in EXT1 and the resulting reduction in HS lead to enhanced Indian
Hedgehog diffusion causing an increase in chondrocyte proliferation and delayed hypertrophic
differentiation.
Dr. Wells serves on the Scientific and Medical Advisory Board of the MHE Research Foundation
Research authored by Dr. Wells
Click the tab and a window will appear.
List of Publications via PubMed
(NIH National Library of Medicine)
Research authored by Dr. Wells
Click the tab and a window will appear.
List of Publications via PubMed
(NIH National Library of Medicine)
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| Dr. Well's research |
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Questions to the Scientific & Medical Advisory Board,
Please use the contact Scientific & Medical Advisory Board Tab or you may email directly
AdvisoryBoard@mheresearchfoundation.org
Please use the contact Scientific & Medical Advisory Board Tab or you may email directly
AdvisoryBoard@mheresearchfoundation.org
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Have a Question or Comments for the Board of Directors,
Please use the contact Board of Directors Tab or General enquiries can be emailed to
Boardofdirectors@mheresearchfoundation.org
Please use the contact Board of Directors Tab or General enquiries can be emailed to
Boardofdirectors@mheresearchfoundation.org
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Disclaimer: While many find the information useful, it is in no way a substitute for professional medical care.
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of medicine. In all cases we recommend that you consult your own physician regarding any course of treatment or medicine.
The information provided here is for educational and informational purposes only. This website does not engage in the practice
of medicine. In all cases we recommend that you consult your own physician regarding any course of treatment or medicine.
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This web page was updated last on 2/20/08, 4:00 pm Eastern time
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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
The MHE Research Foundation is proud to be working with the EuroBoNeT consortium, a European Commission
granted Network of Excellence for studying the pathology and genetics of bone tumors.
granted Network of Excellence for studying the pathology and genetics of bone tumors.
