Richard L. Proia
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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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(Suggest an Edit or Addition)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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What Schools Are Affiliated With Richard L. Proia?
Richard L. Proia is affiliated with the following schools:
