Professor, Internal Medicine, Washington University School of Medicine

Hematology

Stuart Kornfeld is an expert in the field of Hematology. He studied medicine at Washington University School of Medicine in St. Louis. He has 1 awards "Elected Member". Stuart Kornfeld is also a published doctor. He has 102 publication published. The most recent publication is: The rate of internalization of the mannose 6-phosphate/insulin-like growth factor II receptor is enhanced by multivalent ligand binding.

Publications

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• Multiple domains of GlcNAc-1-phosphotransferase mediate recognition of lysosomal enzymes.
• Symbol Nomenclature for Graphical Representations of Glycans.
• Tuberous sclerosis, polycystic kidney disease and mucolipidosis III gamma caused by a microdeletion unmasking a recessive mutation.
• Analysis of Mucolipidosis II/III GNPTAB Missense Mutations Identifies Domains of UDP-GlcNAc:Lysosomal Enzyme GlcNAc-1-Phosphotransferase Involved in Catalytic Function...
• Impact of Genetic Background on Neonatal Lethality of Gga2 Gene-Trap Mice.
• Mislocalization of phosphotransferase as a cause of mucolipidosis III αβ
• The DMAP interaction domain of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase is a substrate recognition module.
• The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.
• The Washington University Medical Scientist Training Program.
• Autoinhibition of the ligand-binding site of GGA1/3 VHS domains by an internal acidic cluster-dileucine motif.
• Neurologic Abnormalities in Mouse Models of the Lysosomal Storage Disorders Mucolipidosis II and Mucolipidosis III γ.
• Rare recessive loss-of-function methionyl-tRNA synthetase mutations presenting as a multi-organ phenotype.
• Lysosomal enzyme binding to mouse P388D1 macrophage membranes lacking the 215-kDa mannose 6-phosphate receptor: evidence for the existence of a second mannose 6-phosph...
• Mannose 6-phosphate/insulin-like growth factor-II receptor targets the urokinase receptor to lysosomes via a novel binding interaction.
• Demonstration of the heterozygous state for I-cell disease and pseudo-Hurler polydystrophy by assay of N-acetylglucosaminylphosphotransferase in white blood cells and ...
• Identification of a rat liver alpha-N-acetylglucosaminyl phosphodiesterase capable of removing "blocking" alpha-N-acetylglucosamine residues from phosphorylated high m...
• Endoplasmic reticulum-associated degradation of mammalian glycoproteins involves sugar chain trimming to Man6-5GlcNAc2.
• The interaction of phosphorylated oligosaccharides and lysosomal enzymes with bovine liver cation-dependent mannose 6-phosphate receptor.
• Lysosomal enzyme phosphorylation in mouse lymphoma cell lines with altered asparagine-linked oligosaccharides.
• Purification and characterization of a cation-dependent mannose 6-phosphate receptor from murine P388D1 macrophages and bovine liver.
• Purification and characterization of UDP-N-acetylgalactosamine: polypeptide N-acetylgalactosaminyltransferase from bovine colostrum and murine lymphoma BW5147 cells.
• The chicken liver cation-independent mannose 6-phosphate receptor lacks the high affinity binding site for insulin-like growth factor II.
• Ligand interactions of the cation-dependent mannose 6-phosphate receptor. Comparison with the cation-independent mannose 6-phosphate receptor.
• Ligand interactions of the cation-independent mannose 6-phosphate receptor. The stoichiometry of mannose 6-phosphate binding.
• Structure and function of the mannose 6-phosphate/insulinlike growth factor II receptors.
• Characterization of UDP-N-acetylglucosamine:glycoprotein N-acetylglucosamine-1-phosphotransferase from Acanthamoeba castellanii.
• Purification of UDP-N-acetylglucosamine:glycoprotein N-acetylglucosamine-1-phosphotransferase from Acanthamoeba castellanii and identification of a subunit of the enzyme.
• Characterization of the signal for rapid internalization of the bovine mannose 6-phosphate/insulin-like growth factor-II receptor.
• A method for [3H]mannose labeling of Asn-linked oligosaccharides on recombinant glycoproteins synthesized in Xenopus oocytes.
• Expression of human cathepsin D in Xenopus oocytes: phosphorylation and intracellular targeting.
• Trafficking of lysosomal enzymes.
• Intracellular movement of two mannose 6-phosphate receptors: return to the Golgi apparatus.
• Cloning and sequence analysis of the cation-independent mannose 6-phosphate receptor.
• Renin, a secretory glycoprotein, acquires phosphomannosyl residues.
• Isolation and characterization of membranes from bovine liver which are highly enriched in mannose 6-phosphate receptors.
• Biochemical dissection of AP-1 recruitment onto Golgi membranes.
• Comparative activity of ADP-ribosylation factor family members in the early steps of coated vesicle formation on rat liver Golgi membranes.
• Analysis of mannose 6-phosphate uncovering enzyme mutations associated with persistent stuttering.
• Wortmannin retards the movement of the mannose 6-phosphate/insulin-like growth factor II receptor and its ligand out of endosomes.
• The biogenesis of lysosomes.
• The biogenesis of the MHC class II compartment in human I-cell disease B lymphoblasts.
• Presidential address: meeting the challenge of change.
• A fascination with sugars.
• Studies of the biosynthesis of the mannose 6-phosphate receptor in receptor-positive and -deficient cell lines.
• The phosphorylation of beta-glucuronidase oligosaccharides in mouse P388D1 cells.
• The mod A mutant of Dictyostelium discoideum is missing the alpha 1,3-glucosidase involved in asparagine-linked oligosaccharide processing.
• Mannose 6-phosphate-independent targeting of lysosomal enzymes in I-cell disease B lymphoblasts.
• The rate of internalization of the mannose 6-phosphate/insulin-like growth factor II receptor is enhanced by multivalent ligand binding.
• Lysosomal enzyme phosphorylation. II. Protein recognition determinants in either lobe of procathepsin D are sufficient for phosphorylation of both the amino and carbox...
• Mutations in the cytoplasmic domain of the 275 kd mannose 6-phosphate receptor differentially alter lysosomal enzyme sorting and endocytosis.
• Generation of a lysosomal enzyme targeting signal in the secretory protein pepsinogen.
• The bovine mannose 6-phosphate/insulin-like growth factor II receptor. Localization of mannose 6-phosphate binding sites to domains 1-3 and 7-11 of the extracytoplasmi...
• AP-2-containing clathrin coats assemble on mature lysosomes.
• Cysteine34 of the cytoplasmic tail of the cation-dependent mannose 6-phosphate receptor is reversibly palmitoylated and required for normal trafficking and lysosomal e...
• The targeting of Lamp1 to lysosomes is dependent on the spacing of its cytoplasmic tail tyrosine sorting motif relative to the membrane.
• Subcellular localization and targeting of cathepsin E.
• Steps in the phosphorylation of the high mannose oligosaccharides of lysosomal enzymes.
• A determinant in the cytoplasmic tail of the cation-dependent mannose 6-phosphate receptor prevents trafficking to lysosomes.
• Evidence for extensive subcellular organization of asparagine-linked oligosaccharide processing and lysosomal enzyme phosphorylation.
• Lysosomal enzyme oligosaccharide phosphorylation in mouse lymphoma cells: specificity and kinetics of binding to the mannose 6-phosphate receptor in vivo.
• Lysosomal enzyme targeting.
• A mannose 6-phosphate-containing N-linked glycopeptide derived from lysosomal acid lipase is bound to MHC class II in B lymphoblastoid cell lines.
• The trans-Golgi network: a late secretory sorting station.
• Vacuolization of mucolipidosis type II mouse exocrine gland cells represents accumulation of autolysosomes.
• Mutation of the COG complex subunit gene COG7 causes a lethal congenital disorder.
• Human mannose 6-phosphate-uncovering enzyme is synthesized as a proenzyme that is activated by the endoprotease furin.
• Cargo-sorting signals promote polymerization of adaptor protein-1 in an Arf-1.GTP-independent manner.
• Mice lacking mannose 6-phosphate uncovering enzyme activity have a milder phenotype than mice deficient for N-acetylglucosamine-1-phosphotransferase activity.
• UDP-GlcNAc:Glycoprotein N-acetylglucosamine-1-phosphotransferase mediates the initial step in the formation of the methylphosphomannosyl residues on the high mannose o...
• Identification and characterization of cells deficient in the mannose 6-phosphate receptor: evidence for an alternate pathway for lysosomal enzyme targeting.
• Cation-dependent mannose 6-phosphate receptor contains two internalization signals in its cytoplasmic domain.
• Proper sorting of the cation-dependent mannose 6-phosphate receptor in endosomes depends on a pair of aromatic amino acids in its cytoplasmic tail.
• Murine UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase lacking the gamma-subunit retains substantial activity toward acid hydrolases.
• Reassessment of the subcellular localization of p63.
• The cytoplasmic tail of the mannose 6-phosphate/insulin-like growth factor-II receptor has two signals for lysosomal enzyme sorting in the Golgi.
• Phosphorylation of Asn-linked oligosaccharides located at novel sites on the lysosomal enzyme cathepsin D.
• Lysosomal enzyme phosphorylation. I. Protein recognition determinants in both lobes of procathepsin D mediate its interaction with UDP-GlcNAc:lysosomal enzyme N-acetyl...
• Expression of the yeast aspartyl protease, proteinase A. Phosphorylation and binding to the mannose 6-phosphate receptor are altered by addition of cathepsin D sequences.
• Mapping and molecular modeling of a recognition domain for lysosomal enzyme targeting.
• Retention of p63 in an ER-Golgi intermediate compartment depends on the presence of all three of its domains and on its ability to form oligomers.
• High-affinity binding of the AP-1 adaptor complex to trans-golgi network membranes devoid of mannose 6-phosphate receptors.
• Functions of the alpha, beta, and gamma subunits of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase.
• A novel mutagenesis strategy identifies distantly spaced amino acid sequences that are required for the phosphorylation of both the oligosaccharides of procathepsin D ...
• Localization of the signal for rapid internalization of the bovine cation-independent mannose 6-phosphate/insulin-like growth factor-II receptor to amino acids 24-29 o...
• A His-Leu-Leu sequence near the carboxyl terminus of the cytoplasmic domain of the cation-dependent mannose 6-phosphate receptor is necessary for the lysosomal enzyme ...
• The cation-independent mannose 6-phosphate receptor binds insulin-like growth factor II.
• The cation-dependent mannose 6-phosphate receptor. Structural requirements for mannose 6-phosphate binding and oligomerization.
• Asparagine-linked oligosaccharides protect Lamp-1 and Lamp-2 from intracellular proteolysis.
• Different domains of the AP-1 adaptor complex are required for Golgi membrane binding and clathrin recruitment.
• Identification of amino acids that modulate mannose phosphorylation of mouse DNase I, a secretory glycoprotein.
• Determination of the structural requirements for palmitoylation of p63.
• The phosphorylation of bovine DNase I Asn-linked oligosaccharides is dependent on specific lysine and arginine residues.
• ADP-ribosylation factor 1 transiently activates high-affinity adaptor protein complex AP-1 binding sites on Golgi membranes.
• The assembly of AP-3 adaptor complex-containing clathrin-coated vesicles on synthetic liposomes.
• Adaptor protein 1-dependent clathrin coat assembly on synthetic liposomes and Golgi membranes.
• Multiple signals regulate trafficking of the mannose 6-phosphate-uncovering enzyme.
• Different domains of mammalian ADP-ribosylation factor 1 mediate interaction with selected target proteins.
• Analysis of Gga Null Mice Demonstrates a Non-Redundant Role for Mammalian GGA2 during Development.
• The cytoplasmic tail of the cation-independent mannose 6-phosphate receptor contains four binding sites for AP-1.
• A splicing mutation in the alpha/beta GlcNAc-1-phosphotransferase gene results in an adult onset form of mucolipidosis III associated with sensory neuropathy and cardi...
• Mucolipidosis III GNPTG missense mutations cause misfolding of the γ subunit of GlcNAc-1-phosphotransferase.

Schools

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• Washington Center School of Medicine

• Barnes Jewish Hospital S Campus

Doctors Specialties

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• Hematology

Accepted Insurances

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• Aetna HMO
• Cigna HMO
• Cigna PPO
• Great West Healthcare-Cigna PPO
• LocalPlus
• Open Access Plus
• Coventry Health Care of Missouri PPO
• HealthLink PPO
• Homestate Health Plan
• Choice Care PPO
• MultiPlan PPO
• PHCS Network PPO
• Choice Plus POS
• Navigate HMO
• Navigate POS
• Options PPO

Awards

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• Elected Member

Education

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• Washington University - Barnes-Jewish Hospital
• Washington University School of Medicine in St. Louis

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