Page last updated: 2024-09-27

Lysosomal Enzyme Disorders, Nervous System

A group of enzymatic disorders affecting the nervous system and to a variable degree the skeletal system, lymphoreticular system, and other organs. The conditions are marked by an abnormal accumulation of catabolic material within lysosomes.

Synonyms(4)

Synonym
Lysosomal Storage Diseases, Nervous System
Nervous System Lysosomal Storage Diseases
Nervous System Lysosomal Enzyme Disorders
Lysosomal Enzyme Disorders, Nervous System

Research

Studies (16)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's14 (87.50)18.1394
2010's2 (12.50)28.8240
2020's0 (0.00)9.53
DrugIndicatedRelationship StrengthStudiesTrials
trimethyloxamine0low10
pk 111950low10
1-anilino-8-naphthalenesulfonate0low10
aspirin0low10
lactose0low10
acetylglucosamine0low10
1-deoxynojirimycin0low20
miglustat0low20
n-acetylglucosaminylasparagine0low10
taurochenodeoxycholic acid0low10
n-acetylneuraminic acid0low20
sphingosine0low10
ursodoxicoltaurine0low10
g(m2) ganglioside0low20
alpha-synuclein0low20
eliglustat tartrate0low10
transforming growth factor beta0low10
g(m3) ganglioside0low10
filipin0low10

Protein Targets (273)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain B, pheromone binding protein0011
Chain A, pheromone binding protein0011
Chain A, JmjC domain-containing histone demethylation protein 3A1001
Chain A, RNA-directed RNA polymerase NS50101
acid sphingomyelinase2002
thioredoxin reductase2002
USP1 protein, partial2002
TDP1 protein3003
vitamin D3 receptor isoform VDRA2002
importin subunit beta-1 isoform 11001
flap endonuclease 11001
serine/threonine-protein kinase PLK11001
snurportin-11001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 11001
GTP-binding nuclear protein Ran isoform 11001
DNA polymerase eta isoform 12002
DNA polymerase iota isoform a (long)4004
urokinase-type plasminogen activator precursor1001
plasminogen precursor1001
urokinase plasminogen activator surface receptor precursor1001
geminin3003
DNA polymerase kappa isoform 12002
fibroblast growth factor 22 isoform 1 precursor0001
Mitogen-activated protein kinase 130101
Beta-lactamase0101
Transthyretin0011
Fatty acid-binding protein, intestinal0112
Fatty acid-binding protein, adipocyte0112
Cyclin-A20101
Cannabinoid receptor 10112
Cyclin-dependent kinase 20112
Choline O-acetyltransferase0101
Mitogen-activated protein kinase 120101
Guanine nucleotide-binding protein G1001
Fatty acid-binding protein 50011
Fatty acid-binding protein 50011
Mitogen-activated protein kinase 110101
Mitogen-activated protein kinase 140101
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE2002
glucocerebrosidase3003
Alpha-mannosidase0101
alpha-galactosidase2002
Trehalase 0202
lysosomal alpha-glucosidase preproprotein1001
Trehalase0101
Maltase-glucoamylase, intestinal0202
Trehalase 0202
Lysosomal acid glucosylceramidase0404
Alpha-galactosidase A0101
Alpha-glucosidase MAL620101
Lactase-phlorizin hydrolase0202
Lysosomal alpha-glucosidase0404
Beta-glucosidase A0101
Sucrase-isomaltase, intestinal0202
Alpha-1B adrenergic receptor0101
Sucrase-isomaltase, intestinal0202
Alpha-1D adrenergic receptor0101
Beta-glucosidase0101
Protein-lysine 6-oxidase0404
Alpha-mannosidase 20202
Glycogen debranching enzyme0202
Glycogen debranching enzyme0101
Alpha-glucosidase MAL320101
Alpha-1A adrenergic receptor0101
Oligo-1,6-glucosidase IMA10101
Alpha-glucosidase MAL120202
Oxysterols receptor LXR-beta0101
Spike glycoprotein1001
Alpha-amylase 0101
Trehalose synthase/amylase TreS0101
Lactase-phlorizin hydrolase 0202
Oxysterols receptor LXR-alpha0101
Neutral alpha-glucosidase AB0202
Ceramide glucosyltransferase0202
Lysosomal acid glucosylceramidase0101
Probable maltase-glucoamylase 20101
Beta-glucosidase 0101
Lysosomal alpha-glucosidase0202
Cytosolic beta-glucosidase0101
Non-lysosomal glucosylceramidase0202
Putative alpha-glucosidase0202
Putative glycosyltransferase WbgO0001
Cytochrome P450 3A40303
Killer cell lectin-like receptor subfamily B member 1A0101
Early activation antigen CD690101
Chain A, TYROSYL-DNA PHOSPHODIESTERASE1001
Chain A, Phospholipase A2 isoform 30011
Chain A, HADH2 protein1001
Chain B, HADH2 protein1001
Chain A, 2-oxoglutarate Oxygenase1001
GTP-binding protein (rab7)0011
ras protein, partial0011
interleukin 81001
GLS protein1001
AR protein2002
aldehyde dehydrogenase 1 family, member A12002
thyroid stimulating hormone receptor1001
hypothetical protein, conserved1001
cytochrome P450 family 3 subfamily A polypeptide 42002
EWS/FLI fusion protein2002
glucocorticoid receptor [Homo sapiens]2002
retinoic acid nuclear receptor alpha variant 12002
estrogen-related nuclear receptor alpha2002
estrogen nuclear receptor alpha2002
cytochrome P450 2D61001
peroxisome proliferator-activated receptor delta1001
Rac1 protein0011
IDH12002
cell division cycle 42 (GTP binding protein, 25kDa), partial0011
euchromatic histone-lysine N-methyltransferase 22002
Bloom syndrome protein isoform 13003
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 11001
muscarinic acetylcholine receptor M11001
lethal factor (plasmid)1001
Prostaglandin-H2 D-isomerase0101
ATP-binding cassette sub-family C member 30101
Multidrug resistance-associated protein 40202
Solute carrier family 22 member 60101
Prostaglandin G/H synthase 1 0101
Prostaglandin G/H synthase 2 0101
Bile salt export pump0204
Epidermal growth factor receptor0101
Carbonic anhydrase 10202
Carbonic anhydrase 20202
Fatty acid-binding protein, liver0101
Integrin beta-30101
Myeloperoxidase0101
Prostaglandin G/H synthase 10101
Seed linoleate 13S-lipoxygenase-10101
Integrin alpha-IIb0101
Glutathione hydrolase 1 proenzyme0101
Prostaglandin G/H synthase 10202
Adenosine receptor A10101
Adenosine receptor A2a0101
Substance-P receptor0101
Prostaglandin G/H synthase 20202
Urotensin-2 receptor0101
4-aminobutyrate aminotransferase, mitochondrial0101
Nuclear receptor ROR-gamma1001
Ras-related protein Rab-2A0011
Gamma-aminobutyric acid receptor subunit alpha-10314
Prostaglandin G/H synthase 20101
Rho-associated protein kinase 20101
Nicotinate phosphoribosyltransferase0101
Solute carrier family 22 member 200101
Solute carrier family 22 member 60101
Canalicular multispecific organic anion transporter 10101
Sigma non-opioid intracellular receptor 10011
Sigma non-opioid intracellular receptor 10011
Chain A, Lectin0011
Chain A, Lectin0011
Chain A, Lectin0011
Chain A, Lectin0011
Chain A, ERYTHRINA CRISTA-GALLI LECTIN0011
Chain A, ERYTHRINA CRISTA-GALLI LECTIN0011
Chain A, cellulase0011
Chain A, cellulase0011
Chain A, Galectin-30011
Chain A, Anti-tumor lectin0101
Galectin-30101
Galectin-90011
Galectin-80011
Beta-galactoside-binding lectin0101
Galectin-10113
Galectin-30112
Galectin-30113
Galectin-70011
Alpha 1,4 galactosyltransferase0001
RAR-related orphan receptor gamma2002
GLI family zinc finger 31001
Sucrase-isomaltase 0101
Ceramide glucosyltransferase0101
Beta-galactosidase0101
Lysosomal alpha-glucosidase0101
Non-lysosomal glucosylceramidase0101
Sialidase-40101
Sialidase-10101
Sialidase-30101
Sialidase-20101
Chain A, Beta-lactamase1001
Chain A, Ferritin light chain2002
endonuclease IV2002
Fumarate hydratase1001
estrogen receptor 2 (ER beta)1001
nuclear receptor subfamily 1, group I, member 31001
regulator of G-protein signaling 41001
retinoid X nuclear receptor alpha1001
farnesoid X nuclear receptor1001
pregnane X nuclear receptor1001
polyprotein1001
Parkin1001
peroxisome proliferator activated receptor gamma1001
vitamin D (1,25- dihydroxyvitamin D3) receptor1001
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a1001
thyroid stimulating hormone receptor1001
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a1001
peripheral myelin protein 22 isoform 11001
prostaglandin E2 receptor EP2 subtype0001
nuclear factor erythroid 2-related factor 2 isoform 21001
thyroid hormone receptor beta isoform 21001
nuclear factor erythroid 2-related factor 2 isoform 11001
neuropeptide S receptor isoform A1001
Gamma-aminobutyric acid receptor subunit pi0213
Gamma-aminobutyric acid receptor subunit beta-10213
Translocator protein0213
Gamma-aminobutyric acid receptor subunit delta0213
Gamma-aminobutyric acid receptor subunit gamma-20213
5-hydroxytryptamine receptor 1A0011
Gamma-aminobutyric acid receptor subunit alpha-50213
Gamma-aminobutyric acid receptor subunit alpha-30213
Gamma-aminobutyric acid receptor subunit gamma-10213
Gamma-aminobutyric acid receptor subunit alpha-20213
Gamma-aminobutyric acid receptor subunit alpha-40213
Gamma-aminobutyric acid receptor subunit gamma-30213
Gamma-aminobutyric acid receptor subunit alpha-60213
Translocator protein0214
Gamma-aminobutyric acid receptor subunit beta-30213
Gamma-aminobutyric acid receptor subunit beta-20213
TAR DNA-binding protein 431001
Nuclear receptor subfamily 1 group I member 3 0101
GABA theta subunit0213
Gamma-aminobutyric acid receptor subunit epsilon0213
ATP-dependent phosphofructokinase1001
Chain A, Cruzipain1001
Luciferase1001
phosphopantetheinyl transferase1001
ATAD5 protein, partial1001
Microtubule-associated protein tau1001
apical membrane antigen 1, AMA11001
thyroid hormone receptor beta isoform a1001
potassium voltage-gated channel subfamily H member 2 isoform d1001
C-X-C chemokine receptor type 5 isoform 10101
DNA polymerase beta1001
mitogen-activated protein kinase 11001
ubiquitin-conjugating enzyme E2 N0101
C-C chemokine receptor type 60101
apelin receptor0101
bcl-2-related protein A10101
streptokinase A precursor0011
lamin isoform A-delta101001
Endoglycoceramidase II 0001
Protein kinase C alpha type0101
Estrogen receptor0011
Protein kinase C delta type0101
Protein kinase C epsilon type0101
Protein kinase C zeta type0101
cAMP-dependent protein kinase catalytic subunit alpha 0101
Protein kinase C gamma type0101
Protein kinase C beta type0101
Estrogen receptor beta0011
Protein kinase C eta type0101
Sphingosine kinase 20101
Sphingosine kinase 10101
Protein kinase C theta type0101
Solute carrier organic anion transporter family member 1A40002
Bile salt export pump0002
Solute carrier organic anion transporter family member 1A50002
Sodium/bile acid cotransporter0002
Solute carrier organic anion transporter family member 1A10002
Ileal sodium/bile acid cotransporter0202
G-protein coupled bile acid receptor 10022
Bile acid receptor0011
Bile salt export pump0001
Phospholipase A20011
Glutamate receptor ionotropic, NMDA 1 0011
Solute carrier organic anion transporter family member 1A20001
Glutamate receptor ionotropic, NMDA 2A 0011
Glutamate receptor ionotropic, NMDA 2B0011
Glutamate receptor ionotropic, NMDA 2C0011
Ectonucleotide pyrophosphatase/phosphodiesterase family member 20101
Glutamate receptor ionotropic, NMDA 2D0011
Glutamate receptor ionotropic, NMDA 3B0011
Glutamate receptor ionotropic, NMDA 3A0011
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]