Stuart Kornfeld

Washington University in St. Louis — United States

Biochemistry, Genetics and Molecular Biology · Life Sciences

104h-index38.7kcitations278works0.82yr avg
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Research Topics

Glycosylation and Glycoproteins Research(146), Cellular transport and secretion(93), Carbohydrate Chemistry and Synthesis(88), Lysosomal Storage Disorders Research(82), Calcium signaling and nucleotide metabolism(43)

Publications278 total

A Genetically-Modified Mouse to Interrogate GPCR-Independent G Protein Signaling with a Synthetic Protein Inhibitor (Abstract ID: 161079)
Journal of Pharmacology and Experimental Therapeutics·2025Open Access
LYSET facilitates integration of both the N- and C-terminal transmembrane helices/cytoplasmic domains of GlcNAc-1-phosphotransferase
Molecular Biology of the Cell·2025· 1 cited
Molecular Insights into the Regulation of GNPTAB by TMEM251
2024· 1 citedOpen Access
Structure of a truncated human GlcNAc-1-phosphotransferase variant reveals the basis for its hyperactivity
Journal of Biological Chemistry·2024· 2 citedOpen Access
Gene therapy with AAV-S1S3 improves disease in mucolipidosis type II mice
Molecular Genetics and Metabolism·2024
Cataloging natural sialic acids and other nonulosonic acids (NulOs), and their representation using the Symbol Nomenclature for Glycans
Glycobiology·2023· 23 citedOpen Access
Structure of the human GlcNAc-1-phosphotransferase αβ subunits reveals regulatory mechanism for lysosomal enzyme glycan phosphorylation
Nature Structural & Molecular Biology·2022· 19 citedOpen Access
Co-expression of S1S3 phosphotransferase in production cell line improves mannose 6-phosphorylation and cellular uptake of alpha--acetylglucosaminidase (Sanfilippo syndrome type B)
Molecular Genetics and Metabolism·2022
Increased phosphorylation of HexM improves lysosomal uptake and potential for managing GM2 gangliosidoses
BBA Advances·2021· 2 citedOpen Access
A weak COPI binding motif in the cytoplasmic tail of SARS‐CoV‐2 spike glycoprotein is necessary for its cleavage, glycosylation, and localization
FEBS Letters·2021· 32 citedOpen Access
Inactivation of the three <i>GGA</i> genes in HeLa cells partially compromises lysosomal enzyme sorting
FEBS Open Bio·2020· 6 citedOpen Access
Disease‐causing missense mutations within the N‐terminal transmembrane domain of GlcNAc‐1‐phosphotransferase impair endoplasmic reticulum translocation or Golgi retention
Human Mutation·2020· 3 citedOpen Access
Updates to the Symbol Nomenclature for Glycans guidelines
Glycobiology·2019· 451 citedOpen Access
Recycling of Golgi glycosyltransferases requires direct binding to coatomer
Proceedings of the National Academy of Sciences·2018· 89 citedOpen Access
A Lifetime of Adventures in Glycobiology
Annual Review of Biochemistry·2018· 33 cited
Engineering of GlcNAc-1-Phosphotransferase for Production of Highly Phosphorylated Lysosomal Enzymes for Enzyme Replacement Therapy
Molecular Therapy — Methods & Clinical Development·2017· 38 citedOpen Access
Role of spacer‐1 in the maturation and function of Glc<scp>NA</scp>c‐1‐phosphotransferase
FEBS Letters·2016· 11 citedOpen Access
Issue Information
Human Mutation·2016Open Access
Mucolipidosis III <i>GNPTG</i> Missense Mutations Cause Misfolding of the γ Subunit of GlcNAc-1-Phosphotransferase
Human Mutation·2016· 5 citedOpen Access
Multiple Domains of GlcNAc-1-phosphotransferase Mediate Recognition of Lysosomal Enzymes
Journal of Biological Chemistry·2016· 47 citedOpen Access
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Frequent Co-authors

Rosalind Kornfeld(6), Ira Tabas(6), Richard D. Cummings(5), Ajit Varki(4), Sondra Schlesinger(4), Hudson H. Freeze(3), Jacques Baenziger(3), Marc L. Reitman(3), Ira Mellman(2), Pradipta Ghosh(2), Nancy Dahms(2), Markus Aebi(2), Nicolle H. Packer(2), Peter H. Seeberger(2), Pamela Stanley(2), Alan G. Darvill(2), Taroh Kinoshita(2), James Prestegard(2), Ronald L. Schnaar(2), Jamey D. Marth(2)