Daniel Ory

Professor of Medicine, Professor of Cell Biology and Physiology, Alan A and Edith L Wolff Professor of Cardiology

  • 12469 Citations
1985 …2020
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Personal profile

Research interests

My lab seeks to understand the basic molecular mechanisms in regulation of cholesterol homeostasis, and the function of the Niemann-Pick C1 (NPC1) protein. A major effort in lab involves genetic screens in cultured cells to identify molecular machinery involved in intracellular cholesterol trafficking. These studies include investigation of the mechanism through which oxygenated forms of cholesterol exert their homeostatic effects, and have led to identification of candidate cholesterol metabolites that are being examined as biomarkers for atherosclerosis and diabetes in human populations. A second area of interest is study of the role of Niemann-Pick C1 (NPC1) in cholesterol homeostasis. The function of NPC1 in cholesterol trafficking in macrophages is being examined in mouse models of atherosclerosis, and the mechanism by which NPC1 loss of function contributes to neuronal cell death is being studied in a mouse knockout model.

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  • 3 Similar Profiles
Type C Niemann-Pick Disease Medicine & Life Sciences
Cholesterol Medicine & Life Sciences
Niemann-Pick Diseases Medicine & Life Sciences
Cyclodextrins Medicine & Life Sciences
Small Nucleolar RNA Medicine & Life Sciences
Homeostasis Medicine & Life Sciences
Lipids Medicine & Life Sciences
Sphingolipids Medicine & Life Sciences

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Research Output 1985 2020

A characterization of Gaucher iPS-derived astrocytes: Potential implications for Parkinson's disease

Aflaki, E., Stubblefield, B. K., McGlinchey, R. P., McMahon, B., Ory, D. S. & Sidransky, E., Feb 2020, In : Neurobiology of Disease. 134, 104647.

Research output: Contribution to journalArticle

Synucleins
Astrocytes
Parkinson Disease
Gaucher Disease
Glucosylceramidase
2 Citations (Scopus)

2-Hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 disease

Davidson, J., Molitor, E., Moores, S., Gale, S. E., Subramanian, K., Jiang, X., Sidhu, R., Kell, P., Zhang, J., Fujiwara, H., Davidson, C., Helquist, P., Melancon, B. J., Grigalunas, M., Liu, G., Salahi, F., Wiest, O., Xu, X., Porter, F. D., Pipalia, N. H. & 6 others, Cruz, D. L., Holson, E. B., Schaffer, J. E., Walkley, S. U., Maxfield, F. R. & Ory, D. S., Oct 2019, In : Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids. 1864, 10, p. 1545-1561 17 p.

Research output: Contribution to journalArticle

Niemann-Pick Diseases
Histone Deacetylase Inhibitors
Cyclodextrins
Pharmacokinetics
Type C Niemann-Pick Disease
3 Citations (Scopus)

Comprehensive behavioral and biochemical outcomes of novel murine models of GM1-gangliosidosis and Morquio syndrome type B

Przybilla, M. J., Ou, L., Tăbăran, A. F., Jiang, X., Sidhu, R., Kell, P. J., Ory, D. S., O'Sullivan, M. G. & Whitley, C. B., Feb 2019, In : Molecular genetics and metabolism. 126, 2, p. 139-150 12 p.

Research output: Contribution to journalArticle

Open Access
Mucopolysaccharidosis IV
GM1 Gangliosidosis
Enzyme activity
Galactosidases
Clustered Regularly Interspaced Short Palindromic Repeats
2 Citations (Scopus)

Diagnosis of niemann-pick C1 by measurement of bile acid biomarkers in archived newborn dried blood spots

Jiang, X., Sidhu, R., Orsini, J. J., Farhat, N. Y., Porter, F. D., Berry-Kravis, E., Schaffer, J. E. & Ory, D. S., Feb 2019, In : Molecular genetics and metabolism. 126, 2, p. 183-187 5 p.

Research output: Contribution to journalArticle

Type C Niemann-Pick Disease
Biomarkers
Bile Acids and Salts
Blood
Newborn Infant
3 Citations (Scopus)

ER–lysosome contacts enable cholesterol sensing by mTORC1 and drive aberrant growth signalling in Niemann–Pick type C

Lim, C. Y., Davis, O. B., Shin, H. R., Zhang, J., Berdan, C. A., Jiang, X., Counihan, J. L., Ory, D. S., Nomura, D. K. & Zoncu, R., Oct 1 2019, In : Nature Cell Biology. 21, 10, p. 1206-1218 13 p.

Research output: Contribution to journalArticle

Cholesterol
Growth
Guanosine
Lysosomes
mechanistic target of rapamycin complex 1