ZFIN Lab: Steet/Flanagan-Steet Lab

ZFIN ID: ZDB-LAB-210330-2

Steet/Flanagan-Steet Lab

PI/Director:	Flanagan-Steet, Heather Steet, Richard
Contact Person:	Flanagan-Steet, Heather
Email:	heatherfs@ggc.org
URL:
Address:	113 Gregor Mendel Circle, Greenwood SC 29646, Greenwood Genetic Center and Clemson University
Country:	United States
Phone:	864-388-1806
Fax:	864-388-1707
Line Designation:	ggc

GENOMIC FEATURES ORIGINATING FROM THIS LAB

Show all 6 genomic features

STATEMENT OF RESEARCH INTERESTS

Our lab utilizes zebrafish to model rare inherited disease to identify the pathogenic cascades that alter tissue development in these disorders. We employ a range of techniques, including systems based approaches, to unravel the complexities of disease.

LAB MEMBERS

Lu, Po-Nien Post-Doc	Wang, Tong Post-Doc	Yu, Seok-ho Research Staff
Jeter, Chelsi Technical Staff	Rimsky, Lynn Technical Staff	Wiggins, Kali Technical Staff
May, Melanie Administrative Staff

ZEBRAFISH PUBLICATIONS OF LAB MEMBERS

Radenkovic, S., Ligezka, A.N., Mokashi, S.S., Driesen, K., Dukes-Rimsky, L., Preston, G., Owuocha, L.F., Sabbagh, L., Mousa, J., Lam, C., Edmondson, A., Larson, A., Schultz, M., Vermeersch, P., Cassiman, D., Witters, P., Beamer, L.J., Kozicz, T., Flanagan-Steet, H., Ghesquière, B., Morava, E. (2023) Tracer metabolomics reveals the role of aldose reductase in glycosylation. Cell reports. Medicine. 4(6):101056

Truong, B.T., Shull, L.C., Lencer, E., Bend, E.G., Field, M., Blue, E.E., Bamshad, M.J., Skinner, C., Everman, D., Schwartz, C.E., Flanagan-Steet, H., Artinger, K.B. (2023) PRDM1 DNA-binding zinc finger domain is required for normal limb development and is disrupted in split hand/foot malformation. Disease models & mechanisms. 16(4):

Klaver, E.J., Dukes-Rimsky, L., Kumar, B., Xia, Z.J., Dang, T., Lehrman, M.A., Angel, P., Drake, R.R., Freeze, H.H., Steet, R., Flanagan-Steet, H. (2021) Protease-dependent defects in N-cadherin processing drive PMM2-CDG pathogenesis. JCI insight. 6(24):

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):

Ates, K.M., Wang, T., Moreland, T., Veeranan-Karmegam, R., Ma, M., Jeter, C., Anand, P., Wenzel, W., Kim, H.G., Wolfe, L.A., Stephen, J.A., Adams, D.R., Markello, T., Tifft, C.J., Settlage, R., Gahl, W.A., Gonsalvez, G.B., Malicdan, M.C., Flanagan-Steet, H., Pan, Y.A. (2020) Deficiency in the endocytic adaptor proteins PHETA1/2 impair renal and craniofacial development. Disease models & mechanisms. 13(5):

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

Flanagan-Steet, H., Fox, M.A., Meyer, D., and Sanes, J.R. (2005) Neuromuscular synapses can form in vivo by incorporation of initially aneural postsynaptic specializations. Development (Cambridge, England). 132(20):4471-4481