ZFIN ID: ZDB-PERS-210330-4
Steet, Richard
Email: rsteet@ggc.org
URL:
Affiliation: Steet/Flanagan-Steet Lab
Address: 113 Gregor Mendel Circle, Greenwood SC 29646, Greenwood Genetic Center and Clemson University
Country: United States
Phone:
Fax:
ORCID ID:


BIOGRAPHY AND RESEARCH INTERESTS


PUBLICATIONS
Yu, S.H., Wang, T., Wiggins, K., Louie, R.J., Merino, E.F., Skinner, C., Cassera, M.B., Meagher, K., Goldberg, P., Rismanchi, N., Chen, D., Lyons, M.J., Flanagan-Steet, H., Steet, R. (2021) Lysosomal cholesterol accumulation contributes to the movement phenotypes associated with NUS1 haploinsufficiency. Genetics in medicine : official journal of the American College of Medical Genetics. 23(7):1305-1314
Lu, P.N., Moreland, T., Christian, C.J., Lund, T.C., Steet, R.A., Flanagan-Steet, H. (2020) Inappropriate cathepsin K secretion promotes its enzymatic activation driving heart and valve malformation. JCI insight. 5(20):
Flanagan-Steet, H., Christian, C., Lu, P.N., Aarnio-Peterson, M., Sanman, L., Archer-Hartmann, S., Azadi, P., Bogyo, M., Steet, R.A. (2018) TGF-ß Regulates Cathepsin Activation during Normal and Pathogenic Development. Cell Reports. 22:2964-2977
Flanagan-Steet, H., Matheny, C., Petrey, A., Parker, J., Steet, R. (2016) Enzyme-specific differences in mannose phosphorylation between GlcNAc-1-phosphotransferase αβ and γ subunit deficient zebrafish support cathepsin proteases as early mediators of mucolipidosis pathology. Biochimica et biophysica acta. General subjects. 1860(9):1845-53
Flanagan-Steet, H., Aarnio, M., Kwan, B., Guihard, P., Petrey, A., Haskins, M., Blanchard, F., Steet, R. (2016) Cathepsin-Mediated Alterations In TGFß-Related Signaling Underlie Disrupted Cartilage and Bone Maturation Associated With Impaired Lysosomal Targeting. Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research. 31(3):535-48
Bammens, R., Mehta, N., Race, V., Foulquier, F., Jaeken, J., Tiemeyer, M., Steet, R., Matthijs, G., Flanagan-Steet, H. (2015) Abnormal Cartilage Development and Altered N-Glycosylation in Tmem165-Deficient Zebrafish Mirrors the Phenotypes Associated with TMEM165-CDG. Glycobiology. 25(6):669-82
Qian, Y., van Meel, E., Flanagan-Steet, H., Yox, A., Steet, R., Kornfeld, S. (2015) Analysis of Mucolipidosis II/III GNPTAB Missense Mutations Identifies Domains of UDP-GlcNAc:Lysosomal Enzyme GlcNAc-1-Phosphotransferase Involved in Catalytic Function and Lysosomal Enzyme Recognition. The Journal of biological chemistry. 290(5):3045-56
Boccuto, L., Aoki, K., Flanagan-Steet, H., Chen, C.F., Fan, X., Bartel, F., Petukh, M., Pittman, A., Saul, R., Chaubey, A., Alexov, E., Tiemeyer, M., Steet, R., and Schwartz, C.E. (2014) A Mutation in a Ganglioside Biosynthetic Enzyme, ST3GAL5, Results in Salt & Pepper Syndrome, a Neurocutaneous Disorder with Altered Glycolipid and Glycoprotein Glycosylation. Human molecular genetics. 23(2):418-33
Qian, Y., Flanagan-Steet, H., van Meel, E., Steet, R., and Kornfeld, S.A. (2013) The DMAP interaction domain of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase is a substrate recognition module. Proceedings of the National Academy of Sciences of the United States of America. 110(25):10246-10251
Flanagan-Steet, H.R., and Steet, R. (2013) "Casting" light on the role of glycosylation during embryonic development: Insights from zebrafish. Glycoconjugate journal. 30(1):33-40
Cline, A., Gao, N., Flanagan-Steet, H., Sharma, V., Rosa, S., Sonon, R., Azadi, P., Sadler, K.C., Freeze, H.H., Lehrman, M.A., and Steet, R. (2012) A Zebrafish Model Of PMM2-CDG Reveals Altered Neurogenesis And A Substrate-Accumulation Mechanism For N-Linked Glycosylation Deficiency. Molecular biology of the cell. 23(21):4175-4187
Petrey, A.C., Flanagan-Steet, H., Johnson, S., Fan, X., De la Rosa, M., Haskins, M.E., Nairn, A.V., Moremen, K.W., and Steet, R. (2012) Excessive activity of cathepsin K is associated with the cartilage defects in a zebrafish model for mucolipidosis II. Disease models & mechanisms. 5(2):177-190
Fan, X., Klein, M., Flanagan-Steet, H.R., and Steet, R. (2010) Selective yolk deposition and mannose phosphorylation of lysosomal glycosidases in zebrafish. The Journal of biological chemistry. 285(43):32946-32953
Flanagan-Steet, H., Sias, C., and Steet, R. (2009) Altered Chondrocyte Differentiation and Extracellular Matrix Homeostasis in a Zebrafish Model for Mucolipidosis II. The American journal of pathology. 175(5):2063-2075

NON-ZEBRAFISH PUBLICATIONS