Richard L. Proia

Q: What Schools Are Affiliated With Richard L. Proia

Richard L. Proia is affiliated with the following schools: University of Virginia, University of California, San Francisco, Brigham Young University, University of Giessen, Harvard University, Ochanomizu University, University of California, Berkeley, University of Oxford, California Institute of Technology, University of Chicago, Stanford University

Richard L. Proia's AcademicInfluence.com Rankings

Richard L. Proia

Biology

#12616

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#16104

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Genetics

#1389

World Rank

#1491

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Molecular Biology

#2066

World Rank

#2098

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Neuroscience

#2130

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#2189

Historical Rank

biology Degrees

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Biology

Richard L. Proia's Degrees

PhD Biochemistry University of California, San Francisco
Masters Molecular Biology Stanford University
Bachelors Biology University of California, Berkeley

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Richard L. Proia's Published Works

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Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author

Published Works

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (2012) (2913)
Lymphocyte egress from thymus and peripheral lymphoid organs is dependent on S1P receptor 1 (2004) (2489)
Edg-1, the G protein-coupled receptor for sphingosine-1-phosphate, is essential for vascular maturation. (2000) (1180)
Lysosomal Glycosphingolipid Recognition by NKT Cells (2004) (1018)
Essential Role for Sphingosine Kinases in Neural and Vascular Development (2005) (721)
The alliance of sphingosine-1-phosphate and its receptors in immunity (2008) (602)
Enhanced insulin sensitivity in mice lacking ganglioside GM3 (2003) (492)
Expression of the Sphingosine 1-Phosphate Receptor, S1P1, on T-cells Controls Thymic Emigration* (2004) (454)
Mice Deficient in Sphingosine Kinase 1 Are Rendered Lymphopenic by FTY720* (2004) (440)
Sphingosine 1-phosphate receptor 1 promotes B cell localization in the splenic marginal zone (2004) (420)
The Sphingosine-1-phosphate Receptors S1P1, S1P2, and S1P3 Function Coordinately during Embryonic Angiogenesis* (2004) (413)
Mouse models of Tay–Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism (1995) (404)
Emerging biology of sphingosine-1-phosphate: its role in pathogenesis and therapy. (2015) (398)
Sphingosine-1-phosphate mobilizes osteoclast precursors and regulates bone homeostasis (2009) (387)
Mice lacking complex gangliosides develop Wallerian degeneration and myelination defects. (1999) (386)
G-protein-coupled receptor S1P1 acts within endothelial cells to regulate vascular maturation. (2003) (378)
Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation. (2000) (368)
Transactivation of Sphingosine-1–Phosphate Receptors by FcɛRI Triggering Is Required for Normal Mast Cell Degranulation and Chemotaxis (2004) (346)
Cyclical modulation of sphingosine-1-phosphate receptor 1 surface expression during lymphocyte recirculation and relationship to lymphoid organ transit (2005) (339)
Immune Cell Regulation and Cardiovascular Effects of Sphingosine 1-Phosphate Receptor Agonists in Rodents Are Mediated via Distinct Receptor Subtypes (2004) (335)
Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin. (1997) (316)
Combinatorial Ganglioside Biosynthesis* (2002) (313)
Sphingosine 1-phosphate receptor regulation of N-cadherin mediates vascular stabilization. (2004) (265)
HDL-bound sphingosine 1-phosphate acts as a biased agonist for the endothelial cell receptor S1P1 to limit vascular inflammation (2015) (247)
Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin. (1999) (241)
Sphingosine-1-Phosphate Induces an Antiinflammatory Phenotype in Macrophages (2008) (237)
Intracellular Role for Sphingosine Kinase 1 in Intestinal Adenoma Cell Proliferation (2006) (236)
Essential role of sphingosine 1-phosphate receptor 2 in pathological angiogenesis of the mouse retina. (2007) (234)
Mice Expressing Only Monosialoganglioside GM3 Exhibit Lethal Audiogenic Seizures* (2001) (221)
The sphingosine kinase-sphingosine-1-phosphate axis is a determinant of mast cell function and anaphylaxis. (2007) (220)
Extracellular export of sphingosine kinase-1a contributes to the vascular S1P gradient. (2006) (219)
Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions. (2005) (211)
Deafness and Stria Vascularis Defects in S1P2 Receptor-null Mice* (2007) (201)
Mice lacking both subunits of lysosomal β–hexosaminidase display gangliosidosis and mucopolysaccharidosis (1996) (195)
Membrane receptors for bacterial toxins (1983) (194)
The sphingosine 1-phosphate receptor S1P2 maintains germinal center B cell homeostasis and promotes niche confinement (2011) (188)
Plasma cell S1P1 expression determines secondary lymphoid organ retention versus bone marrow tropism (2006) (186)
HDL-bound sphingosine 1-phosphate restrains lymphopoiesis and neuroinflammation (2015) (184)
Sphingosine 1-Phosphate Lyase Deficiency Disrupts Lipid Homeostasis in Liver* (2010) (182)
Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice. (2004) (179)
Identification of the protein receptor binding site of botulinum neurotoxins B and G proves the double-receptor concept (2007) (175)
S1P1 receptor overrides regulatory T cell-mediated immune suppression through Akt-mTOR (2009) (171)
Sphingosine-1-phosphate receptors and the development of the vascular system. (2002) (171)
Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease. (1994) (167)
S1P1 receptor directs the release of immature B cells from bone marrow into blood (2010) (167)
Sphingosine-1-phosphate Lyase Deficiency Produces a Pro-inflammatory Response While Impairing Neutrophil Trafficking* (2010) (164)
A novel role of sphingosine 1-phosphate receptor S1pr1 in mouse thrombopoiesis (2012) (159)
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder. (1999) (155)
Depletion of ceramides with very long chain fatty acids causes defective skin permeability barrier function, and neonatal lethality in ELOVL4 deficient mice (2007) (155)
Molecular pathophysiology in Tay-Sachs and Sandhoff diseases as revealed by gene expression profiling. (2002) (153)
Lubricating cell signaling pathways with gangliosides. (2002) (153)
IgE-dependent Activation of Sphingosine Kinases 1 and 2 and Secretion of Sphingosine 1-Phosphate Requires Fyn Kinase and Contributes to Mast Cell Responses* (2006) (148)
Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. (2002) (143)
Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. (1998) (143)
Neutral Ceramidase Encoded by the Asah2 Gene Is Essential for the Intestinal Degradation of Sphingolipids* (2006) (140)
Maternal disturbance in activated sphingolipid metabolism causes pregnancy loss in mice. (2007) (138)
The mutations in Ashkenazi Jews with adult GM2 gangliosidosis, the adult form of Tay-Sachs disease. (1989) (128)
Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation. (2001) (115)
Association of alpha- and beta-subunits during the biosynthesis of beta-hexosaminidase in cultured human fibroblasts. (1984) (115)
The S1P-analog FTY720 differentially modulates T-cell homing via HEV: T-cell-expressed S1P1 amplifies integrin activation in peripheral lymph nodes but not in Peyer patches. (2005) (115)
Sphingosine kinase 1 and sphingosine-1-phosphate receptor 2 are vital to recovery from anaphylactic shock in mice. (2010) (113)
Constitutive expression of the S1P1 receptor in adult tissues. (2004) (106)
Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure. (1998) (103)
Sphingosine kinase 1/S1P receptor signaling axis controls glial proliferation in mice with Sandhoff disease. (2008) (103)
Sphingosine-1-phosphate mobilizes osteoclast precursors and regulates bone homeostasis (2010) (101)
Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse. (2000) (100)
Neurobiological effects of sphingosine 1‐phosphate receptor modulation in the cuprizone model (2011) (97)
Subcellular Origin of Sphingosine 1-Phosphate Is Essential for Its Toxic Effect in Lyase-deficient Neurons* (2009) (93)
Organization of the gene encoding the human beta-hexosaminidase alpha-chain. (1987) (93)
Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease. (2005) (92)
Basal and angiopoietin-1-mediated endothelial permeability is regulated by sphingosine kinase-1. (2008) (90)
Regulation of synaptic strength by sphingosine 1-phosphate in the hippocampus (2010) (89)
Novel Class of Glycosphingolipids Involved in Male Fertility* (2005) (89)
Gene encoding the human beta-hexosaminidase beta chain: extensive homology of intron placement in the alpha- and beta-chain genes. (1988) (88)
Glycosphingolipid functions: insights from engineered mouse models. (2003) (87)
Accumulation of protein‐bound epidermal glucosylceramides in β‐glucocerebrosidase deficient type 2 Gaucher mice (1999) (87)
Essential Requirement for Sphingosine Kinase 2 in a Sphingolipid Apoptosis Pathway Activated by FTY720 Analogues* (2007) (87)
Normal acute and chronic inflammatory responses in sphingosine kinase 1 knockout mice (2006) (85)
Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment. (1997) (83)
Shaping the landscape: metabolic regulation of S1P gradients. (2013) (82)
Erythrocyte-derived sphingosine 1-phosphate is essential for vascular development. (2014) (81)
Synthesis of beta-hexosaminidase in cell-free translation and in intact fibroblasts: an insoluble precursor alpha chain in a rare form of Tay-Sachs disease. (1982) (78)
Sphingosine-1-phosphate receptor 1 reporter mice reveal receptor activation sites in vivo. (2014) (78)
Cerebral organoids derived from Sandhoff disease-induced pluripotent stem cells exhibit impaired neurodifferentiation[S] (2018) (78)
CFTR and sphingolipids mediate hypoxic pulmonary vasoconstriction (2015) (76)
Regulation of limb development by the sphingosine 1-phosphate receptor S1p1/EDG-1 occurs via the hypoxia/VEGF axis. (2004) (68)
S1P1 receptor expression regulates emergence of NKT cells in peripheral tissues (2008) (68)
Physiological Substrates for Human Lysosomal β-Hexosaminidase S* (2002) (67)
Sphingosine-1-Phosphate Enhances Satellite Cell Activation in Dystrophic Muscles through a S1PR2/STAT3 Signaling Pathway (2012) (66)
Kidney Sulfatides in Mouse Models of Inherited Glycosphingolipid Disorders (2002) (64)
Analysis of the glycosylation and phosphorylation of the alpha-subunit of the lysosomal enzyme, beta-hexosaminidase A, by site-directed mutagenesis. (1992) (60)
Binding of soluble myelin‐associated glycoprotein to specific gangliosides induces the association of p75NTR to lipid rafts and signal transduction (2005) (60)
Adipocyte Lipolysis-stimulated Interleukin-6 Production Requires Sphingosine Kinase 1 Activity* (2014) (60)
Sphingosine kinases are not required for inflammatory responses in macrophages. (2016) (55)
Sphingosine-1-Phosphate-Induced Nociceptor Excitation and Ongoing Pain Behavior in Mice and Humans Is Largely Mediated by S1P3 Receptor (2013) (53)
Sphingosine-1-phosphate regulation of mammalian development. (2008) (53)
Inactivation or elimination of potentially trypanolytic, complement-activating immune complexes by pathogenic trypanosomes (1979) (52)
Structure and Expression of the Mouse β-Hexosaminidase Genes, Hexa and Hexb (1994) (49)
Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases. (2000) (48)
Absence of oligodendroglial glucosylceramide synthesis does not result in CNS myelin abnormalities or alter the dysmyelinating phenotype of CGT‐deficient mice (2010) (48)
Mouse Models of Human Lysosomal Diseases (1998) (47)
Simplifying complexity: genetically resculpting glycosphingolipid synthesis pathways in mice to reveal function (2014) (47)
Gangliosides help stabilize the brain (2004) (45)
Faulty association of alpha- and beta-subunits in some forms of beta-hexosaminidase A deficiency. (1984) (45)
Meta-analysis of rare and common exome chip variants identifies S1PR4 and other loci influencing blood cell traits (2016) (45)
Characterization and affinity labeling of the cationic phosphate-binding (nucleotide-binding) peptide located in the receptor-binding region of the B-fragment of diphtheria toxin. (1980) (45)
Ashkenazi-Jewish and non-Jewish adult GM2 gangliosidosis patients share a common genetic defect. (1990) (44)
Analysis of the glycosylation and phosphorylation of the lysosomal enzyme, beta-hexosaminidase B, by site-directed mutagenesis. (1989) (44)
Lysolipid receptor cross-talk regulates lymphatic endothelial junctions in lymph nodes (2019) (44)
Autophagy regulates sphingolipid levels in the liver[S] (2014) (43)
The folding and cell surface expression of CD4 requires glycosylation. (1992) (43)
Roles of AKT and sphingosine kinase in the antiapoptotic effects of bile duct ligation in mouse liver (2005) (43)
Sphingosine kinase regulates the rate of endothelial progenitor cell differentiation. (2009) (42)
cDNA clone for the alpha-chain of human beta-hexosaminidase: deficiency of alpha-chain mRNA in Ashkenazi Tay-Sachs fibroblasts. (1984) (42)
Endothelial S1P1 Signaling Counteracts Infarct Expansion in Ischemic Stroke. (2020) (42)
Neuropathology of mice with targeted disruption of Hexa gene, a model of Tay-Sachs disease (2004) (41)
The GM1 and GM2 Gangliosidoses: Natural History and Progress toward Therapy. (2016) (41)
Human GLB1 knockout cerebral organoids: A model system for testing AAV9-mediated GLB1 gene therapy for reducing GM1 ganglioside storage in GM1 gangliosidosis (2019) (41)
Expression of the ORMDLS, Modulators of Serine Palmitoyltransferase, Is Regulated by Sphingolipids in Mammalian Cells* (2014) (41)
De Novo Sphingolipid Biosynthesis Is Required for Adipocyte Survival and Metabolic Homeostasis* (2017) (40)
Conditional LoxP‐flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis (2005) (39)
Immunoprecipitation and partial characterization of diphtheria toxin-binding glycoproteins from surface of guinea pig cells. (1979) (39)
Embryonic Stem Cells with a Disrupted GD3 Synthase Gene Undergo Neuronal Differentiation in the Absence of b-Series Gangliosides* (1998) (39)
Sphingosine-1-phosphate Phosphatase 1 Regulates Keratinocyte Differentiation and Epidermal Homeostasis*♦ (2013) (38)
The carboxylesterase family exhibits C-terminal sequence diversity reflecting the presence or absence of endoplasmic-reticulum-retention sequences. (1992) (37)
Blood to brain to the rescue. (2004) (36)
Essential Roles of Neutral Ceramidase and Sphingosine in Mitochondrial Dysfunction Due to Traumatic Brain Injury* (2014) (35)
Identification of Domains in Human β-Hexosaminidase That Determine Substrate Specificity* (1996) (34)
Ablation of PPARγ in subcutaneous fat exacerbates age‐associated obesity and metabolic decline (2018) (33)
Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356 (2016) (33)
Mice deficient in all forms of lysosomal beta-hexosaminidase show mucopolysaccharidosis-like pathology. (1997) (33)
The Ormdl genes regulate the sphingolipid synthesis pathway to ensure proper myelination and neurologic function in mice (2019) (33)
Proteolytic processing of the beta-subunit of the lysosomal enzyme, beta-hexosaminidase, in normal human fibroblasts. (1989) (32)
Physiological substrates for human lysosomal beta -hexosaminidase S. (2002) (32)
Diphtheria toxin:receptor interaction. Characterization of the receptor interaction with the nucleotide-free toxin, the nucleotide-bound toxin, and the B-fragment of the toxin. (1981) (31)
A perilous path: the inborn errors of sphingolipid metabolism (2019) (31)
Bioluminescence imaging of G protein-coupled receptor activation in living mice (2017) (30)
Sphingosine-1-phosphate Phosphatase 2 Regulates Pancreatic Islet β-Cell Endoplasmic Reticulum Stress and Proliferation* (2016) (29)
Sphingosine-1-Phosphate and the S1P3 Receptor Initiate Neuronal Retraction via RhoA/ROCK Associated with CRMP2 Phosphorylation (2017) (29)
Tay-Sachs disease in Moroccan Jews: deletion of a phenylalanine in the alpha-subunit of beta-hexosaminidase. (1991) (28)
Identification and rapid detection of three Tay-Sachs mutations in the Moroccan Jewish population. (1992) (28)
B Cell-Specific S1PR1 Deficiency Blocks Prion Dissemination between Secondary Lymphoid Organs (2012) (26)
IGF2BP1 overexpression causes fetal-like hemoglobin expression patterns in cultured human adult erythroblasts (2017) (26)
Synthesis and assembly of a catalytically active lysosomal enzyme, beta-hexosaminidase B, in a cell-free system. (1988) (26)
Brief report G-protein – coupled receptor S 1 P 1 acts within endothelial cells to regulate vascular maturation (2003) (25)
The ORMs interact with transmembrane domain 1 of Lcb1 and regulate serine palmitoyltransferase oligomerization, activity and localization. (2019) (25)
Elevating Endogenous Sphingosine-1-Phosphate (S1P) Levels Improves Endothelial Function and Ameliorates Atherosclerosis in Low Density Lipoprotein Receptor-Deficient (LDL-R−/−) Mice (2018) (23)
Sphingosine 1-phosphate-regulated transcriptomes in heterogenous arterial and lymphatic endothelium of the aorta (2019) (21)
The molecular basis of HEXA mRNA deficiency caused by the most common Tay-Sachs disease mutation. (1995) (21)
Promoters for the human beta-hexosaminidase genes, HEXA and HEXB. (1996) (20)
Sphingosine Kinases Are Not Required for Inflammatory Responses in Macrophages* (2013) (20)
beta-1,4-N-Acetylgalactosaminyltransferase involved in ganglioside synthesis: cDNA sequence, expression, and chromosome mapping of the mouse gene. (1995) (20)
Distribution of enzyme-bearing cells in GM2 gangliosidosis mice: regionally specific pattern of cellular infiltration following bone marrow transplantation (2000) (20)
Early developmental expression of the gene encoding glucosylceramide synthase, the enzyme controlling the first committed step of glycosphingolipid synthesis. (2002) (20)
The role of G protein-coupled receptors in lymphoid malignancies. (2017) (18)
Sphingosine kinase-2 prevents macrophage cholesterol accumulation and atherosclerosis by stimulating autophagic lipid degradation (2019) (18)
Global gene expression in a type 2 Gaucher disease brain. (2004) (17)
The beta-hexosaminidase deficiency disorders: development of a clinical paradigm in the mouse. (1997) (16)
Interaction of diphtheria toxin with phosphorylated molecules (1979) (16)
Usage of Sphingosine Kinase Isoforms in Mast Cells Is Species and/or Cell Type Determined (2013) (16)
Embryonic stem cells with a disrupted GD3 synthase gene undergo neuronal differentiation in the absence of B-series gangliosides (1998) (15)
A genome-wide CRISPR/Cas9 screen reveals that the aryl hydrocarbon receptor stimulates sphingolipid levels (2020) (15)
Structure and expression of the mouse beta-hexosaminidase genes, Hexa and Hexb. (1994) (15)
Evidence for the Involvement of Glu-355 in the Catalytic Action of Human β-Hexosaminidase B* (1997) (14)
Ablation of Sphingosine 1-Phosphate Receptor Subtype 3 Impairs Hippocampal Neuron Excitability In vitro and Spatial Working Memory In vivo (2016) (14)
Diphtheria toxin-binding glycoproteins on hamster cells: candidates for diphtheria toxin receptors (1979) (14)
Molecular basis of heat labile hexosaminidase B among Jews and Arabs (1997) (13)
Synthesis of a human lysosomal enzyme, beta-hexosaminidase B, using the baculovirus expression system. (1990) (12)
Sphingosine‐1‐phosphate can promote mast cell hyper‐reactivity through regulation of contactin‐4 expression (2013) (12)
Correction: Sphingosine-1-Phosphate Enhances Satellite Cell Activation in Dystrophic Muscles through a S1PR2/STAT3 Signaling Pathway (2012) (12)
Activated platelets release sphingosine 1-phosphate and induce hypersensitivity to noxious heat stimuli in vivo (2015) (12)
Studies of lysosomal enzyme biosynthesis in cultured cells. (1983) (12)
S1P4 Regulates Passive Systemic Anaphylaxis in Mice but Is Dispensable for Canonical IgE-Mediated Responses in Mast Cells (2018) (12)
The Complement Regulator Susd4 Influences Nervous-System Function and Neuronal Morphology in Mice (2020) (11)
Glucosylceramide synthase deficiency in the heart compromises β1-adrenergic receptor trafficking (2021) (9)
Murine platelet production is suppressed by S1P release in the hematopoietic niche, not facilitated by blood S1P sensing. (2019) (9)
Sphingosine Kinase-1 Is Essential for Maintaining External/Outer Limiting Membrane and Associated Adherens Junctions in the Aging Retina (2019) (9)
Imaging S1P1 activation in vivo. (2015) (9)
Genetic defects in the sphingolipid degradation pathway and their effects on microglia in neurodegenerative disease. (2020) (9)
Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency. (2000) (7)
Sphingosine-1-Phosphate Enhances α1-Adrenergic Vasoconstriction via S1P2–G12/13–ROCK Mediated Signaling (2019) (6)
Programming to S1PR1+ Endothelial Cells Promotes Restoration of Vascular Integrity (2021) (6)
The mouse gene encoding the GM2 activator protein (Gm2a): cDNA sequence, expression, and chromosome mapping. (1994) (6)
Cell surface immunoglobulin of hamster lymphoid cells. (1979) (5)
DEFECTIVE SYNTHESIS OR MATURATION OF THE α-CHAIN OF β-HEXOSAMINIDASE IN CLASSIC AND VARIANT FORMS OF TAY-SACHS DISEASE (1984) (5)
Sphingosine-1-phosphate enhances satellite cell activation in dystrophic muscles through a S 1 PR 2 / STAT 3 signaling pathway (2016) (5)
Erratum: Sphingosine kinases are not required for inflammatory responses in macrophages (The Journal of Biological Chemistry 288 (2013) 2563-32573) (2016) (3)
Sphingosine Kinase 2 Phosphorylation of FTY720 is Unnecessary for Prevention of Light-Induced Retinal Damage (2019) (3)
Novel HEXA mutation in a Bedouin Tay‐Sachs patient associated with exon skipping and reduced transcript level (1997) (3)
Identification of two lipid phosphatases that regulate sphingosine-1-phosphate cellular uptake and recycling (2022) (3)
Opposing Roles of S1P3 Receptors in Myocardial Function (2020) (3)
Targeting the hexosaminidase genes: mouse models of the GM2 gangliosidoses. (2001) (3)
Cloning the beta-hexosaminidase genes. (2001) (2)
Characterization of a Syrian hamster plasma cell tumor. (1979) (2)
A genome-wide CRISPR/Cas9 screen reveals that the aryl hydrocarbon receptor stimulates sphingolipid levels. (2020) (2)
SPTLC1 Mutations Associated with Early Onset Amyotrophic Lateral Sclerosis (2020) (2)
Erratum: HDL-bound sphingosine 1-phosphate acts as a biased agonist for the endothelial cell receptor S1P to limit vascular inflammation (Science Signaling) (2015) (2)
HEXOSAMINIDASE-PSEUDODEFICIENCY ?. REPLY (1990) (2)
Visualizing S1P-directed cellular egress by intravital imaging. (2014) (2)
A vital role for glycosphingolipid synthesis during development and differentiation. (1999) (1)
Lymphoid Organs Prion Dissemination between Secondary B Cell-Specific S1PR1 Deficiency Blocks (2013) (1)
Unusual cell surface immunoglobulin on a plasma cell tumor. (1979) (1)
Microglial activation and inflammatory reaction preceding neurodegeneration in Sandhoff disease (2001) (1)
Sphingosine-1-Phosphate and the Regulation of Immune Cell Trafficking (2006) (1)
Exacerbation of blood brain barrier dysfunction and brain injury after aneurysmal subarachnoid hemorrhage in endothelial specific S1PR1 knock out mice. (2018) (1)
Heterotypic inter-GPCR ß-arrestin coupling regulates lymphatic endothelial junctional architecture in murine lymph nodes (2018) (1)
substrate deprivation therapy and bone marrow transplantation Enhanced survival in Sandhoff disease mice receiving a combination of (2013) (1)
Murine endothelial serine palmitoyltransferase 1 (SPTLC1) is required for vascular development and systemic sphingolipid homeostasis (2022) (1)
cDNA clone for the a-chain of human f3-hexosaminidase: Deficiency of a-chain mRNA in Ashkenazi Tay-Sachs fibroblasts (1999) (1)
Endothelial sphingosine-1-phosphate receptor 1 deficiency exacerbates brain injury and blood brain barrier dysfunction upon subarachnoid hemorrhage in mice (2018) (1)
709 Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype, neuropathology and ganglioside metabolism (1997) (1)
Receptor-null Mice 2 S1P Deafness and Stria Vascularis Defects in Mechanisms of Signal Transduction: (2014) (0)
Development of isogenic human cerebral organoids with beta-galactosidase deficiency (2016) (0)
Role of Sphingosine 1-phosphate (S1P) in retinal structure and function- lessons learned from mouse models (2020) (0)
Author response: The Ormdl genes regulate the sphingolipid synthesis pathway to ensure proper myelination and neurologic function in mice (2019) (0)
SPTLC1 Mutations Associated with Early Onset Amyotrophic Lateral Sclerosis Impair ORMDL Regulation (2021) (0)
In Memoriam: Roscoe Owen Brady (1923–2016) (2016) (0)
Sphingosine Kinase 2 Phosphorylation of FTY720 is Unnecessary for Prevention of Light-Induced Retinal Damage (2019) (0)
The GM2 gangliosidoses: pathophysiology to therapy (2001) (0)
Tay-Sachs Disease in Moroccan Jews: Deletion of a Phenylalanine in the a-Subunit of P-Hexosaminidase (2006) (0)
nodes but not in Peyer patches amplifies integrin activation in peripheral lymph 1 (2014) (0)
Sphingosine-1-phospate receptor 4 regulates passive systemic anaphylaxis in mice but is dispensable for canonical IgE-mediated responses in mast cells (2018) (0)
vascular maturation G-protein-coupled receptor S1P1 acts within endothelial cells to regulate (2013) (0)
Immunoprecipitation and partial cl toxin-binding glycoproteins from st (cell surface receptors/lentil lectin/lactoperoxidase iodination/m (2016) (0)
Life without gangliosides (2008) (0)
Glb1 knockout mouse model shares natural history with type II GM1 gangliosidosis patients. (2023) (0)
Visualizing Sphingosine-1-Phosphate Receptor 1(S1P1) Signaling During Central Nervous System De- and Remyelination (2021) (0)
Storage of GM2 ganglioside and altered gene expression in infantile Tay-Sachs disease during fetal development has implications for therapeutic timing and efficacy (2022) (0)
Cystic fibrosis, CFTR and hypoxic responses of the lung (2016) (0)
Due to Traumatic Brain Injury Sphingosine in Mitochondrial Dysfunction Essential Roles of Neutral Ceramidase and Lipids (2014) (0)
Cystic fibrosis transmembrane conductance regulator and sphingolipids regulate hypoxic pulmonary vasoconstriction (2015) (0)
Endothelial-derived sphingolipids are required for vascular development and systemic lipid homeostasis (2022) (0)
Immunoglobulins on the surface of hamster lymphocytes. (1981) (0)
Abstract 13667: Programming to S1PR1 + Endothelial Cell Population Promotes the Restoration of Vascular Integrity in vivo (2020) (0)
Sphingosine Kinase-1 Is Essential for Maintaining External/Outer Limiting Membrane and Associated Adherens Junctions in the Aging Retina (2019) (0)
Sphingosine kinase 1 activity Interleukin-6 Production Requires Adipocyte Lipolysis (2014) (0)
SARS‐CoV‐2 ORF3a expression in brain disrupts the autophagy–lysosomal pathway, impairs sphingolipid homeostasis, and drives neuropathogenesis (2023) (0)
88 EFFECT OF THE S1P1 GENE KO ON LIPOPOLYSACCHARIDE-INDUCED MURINE ACUTE LUNG INJURY. (2007) (0)
DeterminedMast Cells Is Species and/or Cell Type Usage of Sphingosine Kinase Isoforms in (2013) (0)
The muscarinic M2-receptor in the chronic asthmatic lung of sphingosine kinase 1 deficient mice (2007) (0)
Elevated chitotriosidase activity in CSF in patients with GM1 and GM2 gangliosidosis: a surrogate marker of disease progression? (2004) (0)
Gene encoding the human 13-hexosaminidase 13 chain : Extensive homology of intron placement in the aand 13-chain genes ( lysosomal enzyme / gene duplication / Sandhoff disease ) (0)
Sphingosine-1-phosphate Lyase Deficiency Produces a Pro-inflammatory Response While Impairing Neutrophil (2011) (0)
Synthesis of j8-hexosaminidase in cell-free translation and in intact fibroblasts: An insoluble precursor (0)
IGF2BP1 Protein Binding of Erythroblast Transcription Factor mRNA: A Cytoplasm-Localized Mechanism for Globin Gene Regulation (2016) (0)
Sleep inspires insight (2004) (0)
Synthesis and Assembly of a Catalytically Active Lysosomal Enzyme , & Hexosaminidase B , in a Cell-free System (0)
Reply to Kappler et al (1990) (0)
Simplifying complexity: genetically resculpting glycosphingolipid synthesis pathways in mice to reveal function (2014) (0)
Bioluminescence imaging of G protein-coupled receptor activation in living mice (2017) (0)
incell-free translation andinintact fibroblasts: Aninsoluble precursor achain inarareformof Tay-Sachs disease (lysosomal enzymes/mannose 6-phosphate/intracellular (1982) (0)
Ablation of Sphingosine Kinase 1 Protects Cornea from Neovascularization in a Mouse Corneal Injury Model (2022) (0)
Gene expression changes in Tay-Sachs disease begin early in fetal brain development. (2023) (0)
Microsoft Word-macrophageSphkKOfinalRev2.docx (2013) (0)
Epidermal Homeostasis Regulates Keratinocyte Differentiation and Sphingosine-1-phosphate Phosphatase 1 Lipids: (2013) (0)
Neutrophilia in severe asthma is reduced in Ormdl3 overexpressing mice (2023) (0)
Biology Revealed by Sphingosine 1‐Phosphate (S1P) Receptor Gene‐Altered Mice (2013) (0)
Targeting GLB1 in mice by CRISPR/Cas9 genome editing: Establishing a novel model for type II GM1 gangliosidosis (2020) (0)

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