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Lysosomal acid glucosylceramidase

A lysosomal acid glucosylceramidase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P04062]

Synonyms

Lysosomal acid GCase;
EC 3.2.1.45;
Acid beta-glucosidase;
Alglucerase;
Beta-glucocerebrosidase;
Beta-GC;
Cholesterol glucosyltransferase;
SGTase;
2.4.1.-;
Cholesteryl-beta-glucosidase;
3.2.1.-;
D-glucosyl-N-acy

Research

Bioassay Publications (32)

TimeframeStudies on this Protein(%)All Drugs %
pre-19900 (0.00)18.7374
1990's2 (6.25)18.2507
2000's7 (21.88)29.6817
2010's21 (65.63)24.3611
2020's2 (6.25)2.80

Compounds (40)

Drugs with Inhibition Measurements

DrugTaxonomyMeasurementAverage (mM)Bioassay(s)Publication(s)
ambroxolHomo sapiens (human)IC504.100011
beta-glucono-1,5-lactoneHomo sapiens (human)Ki30.000011
1-deoxynojirimycinHomo sapiens (human)IC50280.333366
1-deoxynojirimycinHomo sapiens (human)Ki68.333333
miglustatHomo sapiens (human)IC50259.000099
miglustatHomo sapiens (human)Ki116.000011
n-phenylphthalimideHomo sapiens (human)IC50500.000011
N-BenzylphthalimideHomo sapiens (human)IC50500.000011
mor-14Homo sapiens (human)IC5075.500012
1,2,3,4-tetrahydroxy-nor-tropaneHomo sapiens (human)IC503.050022
1,2,3,4-tetrahydroxy-nor-tropaneHomo sapiens (human)Ki3.800011
homonojirimycinHomo sapiens (human)IC501.000011
cyclophellitolHomo sapiens (human)Ki0.152011
calystegine a3Homo sapiens (human)IC5016.000011
calystegine a3Homo sapiens (human)Ki6.300011
miglitolHomo sapiens (human)IC5084.000011
isofagomineHomo sapiens (human)IC500.055544
n-nonyl-1-deoxynojirimycinHomo sapiens (human)IC501.26391515
n-nonyl-1-deoxynojirimycinHomo sapiens (human)Ki0.442977
2-[[4-(4-chloroanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC506.803333
2-[[4-(4-chloroanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)Ki4.230022
4-(benzenesulfonamido)-N-(5-ethyl-1,3,4-thiadiazol-2-yl)benzamideHomo sapiens (human)IC500.601033
4-(benzenesulfonamido)-N-(5-ethyl-1,3,4-thiadiazol-2-yl)benzamideHomo sapiens (human)Ki0.052022
2-[[4-(3-methylanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC505.563333
2-[[4-(3-methylanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)Ki2.780022
N-(1-benzylpiperidin-4-yl)-2-(pyridin-3-yl)quinazolin-4-amineHomo sapiens (human)IC5036.070011
N-[4-methyl-2-(4-morpholinyl)-6-quinolinyl]cyclohexanecarboxamideHomo sapiens (human)IC500.207033
N-[4-methyl-2-(4-morpholinyl)-6-quinolinyl]cyclohexanecarboxamideHomo sapiens (human)Ki0.021022
n-(5-adamantane-1-yl-methoxy-pentyl)deoxynojirimycinHomo sapiens (human)IC500.150022
ogt2378Homo sapiens (human)IC501,000.000011
nnc 26-9100Homo sapiens (human)IC501.605022
2-[[4-[2-[(2-methylpropan-2-yl)oxy]anilino]-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC500.330022
2-[[4-(5-chloro-2-methoxyanilino)-6-(1-piperidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC5031.100022
2-[[4-(4-methylanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC5010.040022
2-[[4-(2-chloroanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC5028.970022
2-[[4-(2-methoxyanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC501.080022
2-[[4-(3-methoxyanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC508.250022
2-[[4-(4-methoxyanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC508.800022
2-[[4-(2-hydroxyethylamino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]phenolHomo sapiens (human)IC502.000022
2-[[4-(2-phenoxyanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC5021.670022
2-[[4-(1-pyrrolidinyl)-6-[2-(trifluoromethoxy)anilino]-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC503.340022
2-[[4-(5-chloro-2-ethoxyanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC500.960022
2-[[4-(5-chloro-2-propan-2-yloxyanilino)-6-(1-pyrrolidinyl)-1,3,5-triazin-2-yl]amino]ethanolHomo sapiens (human)IC503.440022

Drugs with Activation Measurements

DrugTaxonomyMeasurementAverage (mM)Bioassay(s)Publication(s)
1-(10h-phenothiazin-2-yl)ethanoneHomo sapiens (human)EC5050.000010
1-(1-benzimidazolyl)-3-(1-cyclohex-3-enylmethoxy)-2-propanolHomo sapiens (human)EC5050.000010
2-(2-phenylethylthio)-3-pyridinecarboxylic acidHomo sapiens (human)EC5050.000010
4-(2-furanylmethyl)-3-(phenylmethyl)-1H-1,2,4-triazole-5-thioneHomo sapiens (human)EC5050.000010
tamoxifenHomo sapiens (human)EC502.500011
4-oxido-3-(4-phenoxyphenyl)-4a,5,6,7,8,8a-hexahydroquinoxalin-1-ium 1-oxideHomo sapiens (human)EC5050.000010

Drugs with Other Measurements

DrugTaxonomyMeasurementAverage (mM)Bioassay(s)Publication(s)
isofagomineHomo sapiens (human)INH0.110011
4-(benzenesulfonamido)-N-(5-ethyl-1,3,4-thiadiazol-2-yl)benzamideHomo sapiens (human)AC500.070011

Enables

This protein enables 7 target(s):

TargetCategoryDefinition
galactosylceramidase activitymolecular functionCatalysis of the reaction: D-galactosyl-N-acylsphingosine + H2O = D-galactose + N-acylsphingosine. [EC:3.2.1.46]
glucosylceramidase activitymolecular functionCatalysis of the reaction: D-glucosyl-N-acylsphingosine + H2O = D-glucose + N-acylsphingosine. [EC:3.2.1.45]
signaling receptor bindingmolecular functionBinding to one or more specific sites on a receptor molecule, a macromolecule that undergoes combination with a hormone, neurotransmitter, drug or intracellular messenger to initiate a change in cell function. [GOC:bf, GOC:ceb, ISBN:0198506732]
scavenger receptor bindingmolecular functionBinding to scavenger receptors, a family of proteins that are expressed on myeloid cells and are involved in the uptake of effete cellular components and foreign particles. [GOC:ceb]
protein bindingmolecular functionBinding to a protein. [GOC:go_curators]
glucosyltransferase activitymolecular functionCatalysis of the transfer of a glucosyl group to an acceptor molecule, typically another carbohydrate or a lipid. [ISBN:0198506732]
steryl-beta-glucosidase activitymolecular functionCatalysis of the reaction: cholesteryl-beta-D-glucoside + H2O = D-glucose + cholesterol. [EC:3.2.1.104, RHEA:11956]

Located In

This protein is located in 7 target(s):

TargetCategoryDefinition
lysosomecellular componentA small lytic vacuole that has cell cycle-independent morphology found in most animal cells and that contains a variety of hydrolases, most of which have their maximal activities in the pH range 5-6. The contained enzymes display latency if properly isolated. About 40 different lysosomal hydrolases are known and lysosomes have a great variety of morphologies and functions. [GOC:mah, ISBN:0198506732]
lysosomal membranecellular componentThe lipid bilayer surrounding the lysosome and separating its contents from the cell cytoplasm. [GOC:ai]
endoplasmic reticulumcellular componentThe irregular network of unit membranes, visible only by electron microscopy, that occurs in the cytoplasm of many eukaryotic cells. The membranes form a complex meshwork of tubular channels, which are often expanded into slitlike cavities called cisternae. The ER takes two forms, rough (or granular), with ribosomes adhering to the outer surface, and smooth (with no ribosomes attached). [ISBN:0198506732]
Golgi apparatuscellular componentA membrane-bound cytoplasmic organelle of the endomembrane system that further processes the core oligosaccharides (e.g. N-glycans) added to proteins in the endoplasmic reticulum and packages them into membrane-bound vesicles. The Golgi apparatus operates at the intersection of the secretory, lysosomal, and endocytic pathways. [ISBN:0198506732]
trans-Golgi networkcellular componentThe network of interconnected tubular and cisternal structures located within the Golgi apparatus on the side distal to the endoplasmic reticulum, from which secretory vesicles emerge. The trans-Golgi network is important in the later stages of protein secretion where it is thought to play a key role in the sorting and targeting of secreted proteins to the correct destination. [GOC:vw, ISBN:0815316194]
lysosomal lumencellular componentThe volume enclosed within the lysosomal membrane. [GOC:jl, PMID:15213228]
extracellular exosomecellular componentA vesicle that is released into the extracellular region by fusion of the limiting endosomal membrane of a multivesicular body with the plasma membrane. Extracellular exosomes, also simply called exosomes, have a diameter of about 40-100 nm. [GOC:BHF, GOC:mah, GOC:vesicles, PMID:15908444, PMID:17641064, PMID:19442504, PMID:19498381, PMID:22418571, PMID:24009894]

Involved In

This protein is involved in 50 target(s):

TargetCategoryDefinition
glucosylceramide catabolic processbiological processThe chemical reactions and pathways resulting in the breakdown of glucosylceramides, any compound formed by the replacement of the glycosidic hydroxyl group of a cyclic form of glucose by a ceramide group. [GOC:ai]
autophagybiological processThe cellular catabolic process in which cells digest cellular materials, such as organelles and other macromolecular constituents, or non-self materials such as intracellular pathogens. Autophagy serves to provide essential nutrients under conditions of cellular stress; or can remodel intracellular structures during cell differentiation. [GOC:autophagy, ISBN:0198547684, PMID:11099404, PMID:29455577, PMID:9412464]
lysosome organizationbiological processA process that is carried out at the cellular level which results in the assembly, arrangement of constituent parts, or disassembly of a lysosome. A lysosome is a cytoplasmic, membrane-bounded organelle that is found in most animal cells and that contains a variety of hydrolases. [GOC:mah]
cholesterol metabolic processbiological processThe chemical reactions and pathways involving cholesterol, cholest-5-en-3 beta-ol, the principal sterol of vertebrates and the precursor of many steroids, including bile acids and steroid hormones. It is a component of the plasma membrane lipid bilayer and of plasma lipoproteins and can be found in all animal tissues. [ISBN:0198506732]
determination of adult lifespanbiological processThe pathways that regulate the duration of the adult phase of the life-cycle of an animal. [PMID:25561524, PMID:273723695, PMID:3424805]
cellular response to starvationbiological processAny process that results in a change in state or activity of a cell (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of deprivation of nourishment. [GOC:jl]
response to pHbiological processAny process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a pH stimulus. pH is a measure of the acidity or basicity of an aqueous solution. [GOC:jl, Wikipedia:PH]
microglia differentiationbiological processThe process in which a relatively unspecialized cell acquires specialized features of a microglial cell. Microglia are glial cells that act as the immune cells of the central nervous system. They form part of the supporting structure of this system. [GOC:ef]
regulation of macroautophagybiological processAny process that modulates the frequency, rate or extent of macroautophagy. [GOC:krc]
antigen processing and presentationbiological processThe process in which an antigen-presenting cell expresses antigen (peptide or lipid) on its cell surface in association with an MHC protein complex. [GO_REF:0000022, GOC:add, ISBN:0781735149, PMID:15771591, PMID:15928678]
lipid storagebiological processThe accumulation and maintenance in cells or tissues of lipids, compounds soluble in organic solvents but insoluble or sparingly soluble in aqueous solvents. Lipid reserves can be accumulated during early developmental stages for mobilization and utilization at later stages of development. [GOC:dph, GOC:mah, GOC:tb, PMID:11102830]
cerebellar Purkinje cell layer formationbiological processThe process that gives rise to the cerebellar Purkinje cell layer. This process pertains to the initial formation of a structure from unspecified parts. The Purkinje cell layer lies just underneath the molecular layer of the cerebellar cortex. It contains the neuronal cell bodies of the Purkinje cells that are arranged side by side in a single layer. Candelabrum interneurons are vertically oriented between the Purkinje cells. Purkinje neurons are inhibitory and provide the output of the cerebellar cortex through axons that project into the white matter. Extensive dendritic trees from the Purkinje cells extend upward in a single plane into the molecular layer where they synapse with parallel fibers of granule cells. [GO_REF:0000021, GOC:cls, GOC:dgh, GOC:dph, GOC:jid, ISBN:0838580343]
pyramidal neuron differentiationbiological processThe process in which a neuroblast or one of its progeny commits to a pyramidal neuron fate, migrates from the ventricular zone to the appropriate layer in the cortex and develops into a mature neuron. [GO_REF:0000021, GOC:cls, GOC:dgh, GOC:dph, GOC:jid]
respiratory electron transport chainbiological processA process in which a series of electron carriers operate together to transfer electrons from donors such as NADH and FADH2 to any of several different terminal electron acceptors to generate a transmembrane electrochemical gradient. [GOC:mtg_electron_transport, ISBN:0716720094]
termination of signal transductionbiological processThe signaling process in which signal transduction is brought to an end rather than being reversibly modulated. [GOC:mtg_signal]
lipid glycosylationbiological processCovalent attachment of a glycosyl residue to a lipid molecule. [GOC:mah]
negative regulation of protein-containing complex assemblybiological processAny process that stops, prevents, or reduces the frequency, rate or extent of protein complex assembly. [GOC:mah]
regulation of TOR signalingbiological processAny process that modulates the frequency, rate or extent of TOR signaling. [GOC:mah]
positive regulation of proteasomal ubiquitin-dependent protein catabolic processbiological processAny process that activates or increases the frequency, rate or extent of the breakdown of a protein or peptide by hydrolysis of its peptide bonds, initiated by the covalent attachment of ubiquitin, and mediated by the proteasome. [GOC:mah]
negative regulation of interleukin-6 productionbiological processAny process that stops, prevents, or reduces the frequency, rate, or extent of interleukin-6 production. [GOC:mah]
T cell differentiation in thymusbiological processThe process in which a precursor cell type acquires the specialized features of a T cell via a differentiation pathway dependent upon transit through the thymus. [GOC:add, ISBN:0781735149]
response to testosteronebiological processAny process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a testosterone stimulus. [GOC:sl]
positive regulation of protein dephosphorylationbiological processAny process that activates or increases the frequency, rate or extent of removal of phosphate groups from a protein. [GOC:bf]
proteasome-mediated ubiquitin-dependent protein catabolic processbiological processThe chemical reactions and pathways resulting in the breakdown of a protein or peptide by hydrolysis of its peptide bonds, initiated by the covalent attachment of ubiquitin, and mediated by the proteasome. [GOC:go_curators]
positive regulation of protein-containing complex disassemblybiological processAny process that activates or increases the frequency, rate or extent of protein complex disassembly, the disaggregation of a protein complex into its constituent components. [GOC:jl]
negative regulation of MAP kinase activitybiological processAny process that stops, prevents, or reduces the frequency, rate or extent of MAP kinase activity. [GOC:dph, GOC:go_curators]
negative regulation of neuron apoptotic processbiological processAny process that stops, prevents, or reduces the frequency, rate or extent of cell death by apoptotic process in neurons. [GOC:go_curators, GOC:mtg_apoptosis]
response to estrogenbiological processAny process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of stimulus by an estrogen, C18 steroid hormones that can stimulate the development of female sexual characteristics. [GOC:jl, ISBN:0198506732]
sphingosine biosynthetic processbiological processThe chemical reactions and pathways resulting in the formation of sphingosine (sphing-4-enine), trans-D-erytho-2-amino-octadec-4-ene-1,3-diol, a long chain amino diol sphingoid base that occurs in most sphingolipids in animal tissues. [GOC:ma, ISBN:0198506732]
ceramide biosynthetic processbiological processThe chemical reactions and pathways resulting in the formation of ceramides, any N-acylated sphingoid. [GOC:ai]
cell maturationbiological processThe cellular developmental process, independent of morphogenetic (shape) change, that is required for a specific cell to attain its fully functional state. [GOC:go_curators]
brain morphogenesisbiological processThe process in which the anatomical structures of the brain are generated and organized. The brain is one of the two components of the central nervous system and is the center of thought and emotion. It is responsible for the coordination and control of bodily activities and the interpretation of information from the senses (sight, hearing, smell, etc.). [GOC:dgh, GOC:jid]
homeostasis of number of cellsbiological processAny biological process involved in the maintenance of the steady-state number of cells within a population of cells. [GOC:isa_complete]
negative regulation of inflammatory responsebiological processAny process that stops, prevents, or reduces the frequency, rate or extent of the inflammatory response. [GOC:ai]
neuromuscular processbiological processAny process pertaining to the functions of the nervous and muscular systems of an organism. [GOC:ai]
neuron apoptotic processbiological processAny apoptotic process in a neuron, the basic cellular unit of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the nervous system. [CL:0000540, GOC:mtg_apoptosis]
establishment of skin barrierbiological processEstablishment of the epithelial barrier, the functional barrier in the skin that limits its permeability. [GOC:dph]
microglial cell proliferationbiological processThe expansion of a microglial cell population by cell division. [GOC:dph, PMID:17344397]
motor behaviorbiological processThe specific neuromuscular movement of a single organism in response to external or internal stimuli. [GOC:bf, GOC:PARL, PMID:25318560]
cellular response to tumor necrosis factorbiological processAny process that results in a change in state or activity of a cell (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a tumor necrosis factor stimulus. [GOC:mah]
hematopoietic stem cell proliferationbiological processThe expansion of a hematopoietic stem cell population by cell division. A hematopoietic stem cell is a stem cell from which all cells of the lymphoid and myeloid lineages develop. [CL:0000037, GOC:add, GOC:BHF, GOC:mah, GOC:rl]
response to dexamethasonebiological processAny process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a dexamethasone stimulus. [GOC:mah, GOC:yaf]
lymphocyte migrationbiological processThe movement of a lymphocyte within or between different tissues and organs of the body. [CL:0000542, GOC:BHF, GOC:mah]
response to thyroid hormonebiological processA change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a thyroid hormone stimulus. [GOC:sjw, PMID:9916872]
beta-glucoside catabolic processbiological processThe chemical reactions and pathways resulting in the breakdown of beta-glucoside. [GOC:TermGenie, GOC:yaf, PMID:15205427, PMID:16390337, PMID:8990303, Unipathway:UPA00237]
positive regulation of protein lipidationbiological processAny process that activates or increases the frequency, rate or extent of protein lipidation. [GO_REF:0000058, GOC:rph, GOC:TermGenie, PMID:21909394]
positive regulation of neuronal action potentialbiological processAny process that activates or increases the frequency, rate or extent of neuronal action potential. [GO_REF:0000058, GOC:TermGenie, PMID:25126967]
positive regulation of autophagy of mitochondrion in response to mitochondrial depolarizationbiological processAny process that activates or increases the frequency, rate or extent of autophagy of the mitochondrion in response to mitochondrial depolarization. [GO_REF:0000058, GOC:pad, GOC:PARL, GOC:TermGenie, PMID:22020285]
autophagosome organizationbiological processA process that is carried out at the cellular level which results in the assembly, arrangement of constituent parts, or disassembly of an autophagosome. [GOC:bf, GOC:PARL, GOC:TermGenie, PMID:22186024]
regulation of lysosomal protein catabolic processbiological processAny process that modulates the frequency, rate or extent of lysosomal protein catabolic process. [GO_REF:0000058, GOC:bf, GOC:PARL, GOC:TermGenie, PMID:23499937]