Research
Search
Genes / Clones
Expression
Mutants / Tg
Antibodies
Anatomy / GO / Human Disease / Chemical
Publications
Data Mining
Downloads
Data Model
AllianceMine
BioMart
Genomics
BLAST
ZFIN
Ensembl
NCBI
UCSC
Genome Browsers
ZFIN
Ensembl
Vega
GRC
UCSC
NCBI
Resources
Zebrafish Genomics
Other Genome Databases
Resources
General
The Zebrafish Book
Protocol Wiki
Antibody Wiki
Anatomy Atlases
Resources for Students and Educators
Zebrafish Programs
ZF-Health
Husbandry Resources
More...
Resource Centers
Zebrafish International Resource Center (ZIRC)
China Zebrafish Resource Center (CZRC)
European Zebrafish Resource Center (EZRC)
Community
Announcements
News
Meetings
Jobs
Alliance Community Forum
Search
People
Labs
Companies
Societies
International Zebrafish Society (IZFS)
Zebrafish Disease Models Society (ZDMS)
Genetics Society of America (GSA)
Zebrafish Husbandry Association
Support
Nomenclature
Nomenclature Conventions
Line Designations
Wild-Type Lines
Submit a Proposed Gene Name
Submit a Proposed Mutant/Tg Line Name
Publications
Guidelines for Authors
Zebrashare
Citing ZFIN
Using ZFIN
Help & Tips
Glossary
Single Box Search Help
Submit Data
Terms of Use
About Us
About ZFIN
Contact Information
Statistics
Committees
Jobs at ZFIN
Sign In
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
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):
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
Your Input Welcome
Your Input Welcome
We welcome your input and comments. Please use this form to recommend updates to the information in ZFIN. We appreciate as much detail as possible and references as appropriate. We will review your comments promptly.
Please check the highlighted fields and try again.
Name:
Institution:
Email address:
Please leave blank:
Subject:
Comments:
Send your comments
Thank you for submitting comments. Your input has been emailed to ZFIN curators who may contact you if additional information is required.
Oops. Something went wrong. Please try again later.