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Outputs (12)

Sost haploinsufficiency provokes peracute lethal cardiac tamponade without rescuing the osteopenia in a mouse model of excess glucocorticoids (2019)
Journal Article
Javaheri, B., Herbert, E., Hopkinson, M., Al-Jazzar, A., & Pitsillides, A. A. (2019). Sost haploinsufficiency provokes peracute lethal cardiac tamponade without rescuing the osteopenia in a mouse model of excess glucocorticoids. American Journal of Pathology, 189(4), 753-761. https://doi.org/10.1016/j.ajpath.2018.12.007

Glucocorticoid-induced secondary osteoporosis is the most predictable side-effect of this anti-inflammatory. One of the main mechanisms by which glucocorticoids achieve such deleterious outcome in bone is by antagonizing Wnt/β-catenin signalling. Scl... Read More about Sost haploinsufficiency provokes peracute lethal cardiac tamponade without rescuing the osteopenia in a mouse model of excess glucocorticoids.

Hypomorphic conditional deletion of E11/Podoplanin reveals a role in osteocyte dendrite elongation (2017)
Journal Article
Staines, K. A., Javaheri, B., Hohenstein, P., Fleming, R., Ikpegbu, E., Unger, E., Hopkinson, M., Buttle, D. J., Pitsillides, A. A., & Farquharson, C. (2017). Hypomorphic conditional deletion of E11/Podoplanin reveals a role in osteocyte dendrite elongation. Journal of Cellular Physiology, 232(11), 3006-3019. https://doi.org/10.1002/jcp.25999

The transmembrane glycoprotein E11/Podoplanin (Pdpn) has been implicated in the initial stages of osteocyte differentiation. However, its precise function and regulatory mechanisms are still unknown. Due to the known embryonic lethality induced by gl... Read More about Hypomorphic conditional deletion of E11/Podoplanin reveals a role in osteocyte dendrite elongation.

Dmp1 Promoter-Driven Diphtheria Toxin Receptor Transgene Expression Directs Unforeseen Effects in Multiple Tissues (2017)
Journal Article
Al-Jazzar, A., Javaheri, B., Prideaux, M., Boyde, A., Scudamore, C. L., Cherifi, C., Hay, E., Hopkinson, M., Boyd, M., Cohen-Solal, M., Farquharson, C., & Pitsillides, A. A. (2017). Dmp1 Promoter-Driven Diphtheria Toxin Receptor Transgene Expression Directs Unforeseen Effects in Multiple Tissues. International Journal of Molecular Sciences, 18(1), https://doi.org/10.3390/ijms18010029

Mice harbouring a dentin matrix protein 1 (Dmp1) promoter-driven human diphtheria toxin (DT) receptor (HDTR) transgene (Tg) have recently been used to attain targeted ablation of osteocytes by diphtheria toxin (DT) treatment in order to define osteoc... Read More about Dmp1 Promoter-Driven Diphtheria Toxin Receptor Transgene Expression Directs Unforeseen Effects in Multiple Tissues.

Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window? (2016)
Journal Article
Hildyard, J. C. W., Lacey, E., Booler, H., Hopkinson, M., Wells, D. J., & Brown, S. C. (2016). Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?. PLoS ONE, 11(7), e0159853. https://doi.org/10.1371/journal.pone.0159853

LARGE is a glycosyltransferase involved in glycosylation of α-dystroglycan (α-DG). Absence of this protein in the LARGEmyd mouse results in α-DG hypoglycosylation, and is associated with central nervous system abnormalities and progressive muscular d... Read More about Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?.

Degree of Cajal-Retzius cell mislocalisation correlates with the severity of structural brain defects in mouse models of dystroglycanopathy (2016)
Journal Article
Booler, H., Williams, J., Hopkinson, M., & Brown, S. C. (2016). Degree of Cajal-Retzius cell mislocalisation correlates with the severity of structural brain defects in mouse models of dystroglycanopathy. Brain Pathology, 26(4), 465-478. https://doi.org/10.1111/bpa.12306

The secondary dystroglycanopathies are characterized by the hypoglycosylation of alpha dystroglycan, and are associated with mutations in at least 18 genes that act on the glycosylation of this cell surface receptor rather than the Dag1 gene itself.... Read More about Degree of Cajal-Retzius cell mislocalisation correlates with the severity of structural brain defects in mouse models of dystroglycanopathy.

Prenatal muscle development in a mouse model for the secondary dystroglycanopathies (2016)
Journal Article
Kim, J., Hopkinson, M., Kavishwar, M., Fernandez-Fuente, M., & Brown, S. C. (2016). Prenatal muscle development in a mouse model for the secondary dystroglycanopathies. Skeletal Muscle, 6(3), https://doi.org/10.1186/s13395-016-0073-y

The defective glycosylation of α-dystroglycan is associated with a group of muscular dystrophies that are collectively referred to as the secondary dystroglycanopathies. Mutations in the gene encoding fukutin-related protein (FKRP) are one of the mos... Read More about Prenatal muscle development in a mouse model for the secondary dystroglycanopathies.

Endochondral growth defect and deployment of transient chondrocyte behaviours underlie osteoarthritis onset in a natural murine model (2016)
Journal Article
Staines, K. A., Madi, K., Mirczuk, S. M., Parker, S., Burleigh, A., Poulet, B., Hopkinson, M., Bodey, A. J., Fowkes, R. C., Farquharson, C., Lee, P. D., & Pitsillides, A. A. (2016). Endochondral growth defect and deployment of transient chondrocyte behaviours underlie osteoarthritis onset in a natural murine model. https://doi.org/10.1002/art.39508

Excessive growth hormone expression in male GH transgenic mice adversely alters bone architecture and mechanical strength (2015)
Journal Article
Lim, S., Marenzana, M., Hopkinson, M., List, E., Kopchick, J., Pereira, M., Javaheri, B., Roux, J., Chavassieux, P., Korbonits, M., & Chenu, C. (2015). Excessive growth hormone expression in male GH transgenic mice adversely alters bone architecture and mechanical strength. Endocrinology, 156(4), 1362-1371. https://doi.org/10.1210/en.2014-1572

Patients with acromegaly have a higher prevalence of vertebral fractures despite normal bone mineral density (BMD), suggesting that GH overexpression has adverse effects on skeletal architecture and strength. We used giant bovine GH (bGH) transgenic... Read More about Excessive growth hormone expression in male GH transgenic mice adversely alters bone architecture and mechanical strength.