Page last updated: 2024-09-27

Unverricht-Lundborg Syndrome

An autosomal recessive condition characterized by recurrent myoclonic and generalized seizures, ATAXIA, slowly progressive intellectual deterioration, DYSARTHRIA, and intention tremor. Myoclonic seizures are severe and continuous, and tend to be triggered by movement, stress, and sensory stimuli. The age of onset is between 8 and 13 years, and the condition is relatively frequent in the Baltic region, especially Finland. (From Menkes, Textbook of Child Neurology, 5th ed, pp109-110)

Synonyms(1)

Synonym
Unverricht-Lundborg Syndrome

Research

Studies (40)

TimeframeStudies, This Condition (%)All Conditions %
pre-19901 (2.50)23.3326
1990's0 (0.00)12.5806
2000's18 (45.00)18.1394
2010's17 (42.50)28.8240
2020's4 (10.00)9.53
DrugIndicatedRelationship StrengthStudiesTrials
gamma-aminobutyric acid0low20
choline0low20
creatine0low20
kynurenine0low10
phenytoin0low10
hydroxyindoleacetic acid0low10
chloral hydrate0low10
clonazepam0medium21
diethyl pyrocarbonate0low10
valproic acid0medium92
lamotrigine0medium11
mephenesin0low10
phenobarbital0medium11
piracetam0medium72
potassium chloride0low10
primidone0low10
propidium0low10
zonisamide0medium41
aspartic acid0low20
tryptophan0low10
dimethyl sulfate0low10
isoxazoles0medium41
thiazoles0low10
kainic acid0low10
acetylcysteine0low20
thioflavin t0low10
glutamic acid0low10
n-acetylaspartic acid0low10
D-fructopyranose0medium11
raclopride0low10
levetiracetam0medium72
topiramate0medium11
perampanel0low40
alpha-synuclein0low10
losartan potassium0low10
guanosine0low10

Protein Targets (493)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain A, JmjC domain-containing histone demethylation protein 3A2002
Chain A, 2-oxoglutarate Oxygenase7007
Chain A, ATP-DEPENDENT DNA HELICASE Q13003
acetylcholinesterase3003
dopamine D1 receptor2002
15-lipoxygenase, partial1001
pregnane X receptor3003
phosphopantetheinyl transferase7007
RAR-related orphan receptor gamma2002
USP1 protein, partial4004
NFKB1 protein, partial2002
Microtubule-associated protein tau5005
Thrombopoietin1001
AR protein6006
regulator of G-protein signaling 46006
glucocorticoid receptor [Homo sapiens]5005
retinoic acid nuclear receptor alpha variant 15005
retinoid X nuclear receptor alpha4004
farnesoid X nuclear receptor3003
estrogen nuclear receptor alpha7007
vitamin D (1,25- dihydroxyvitamin D3) receptor3003
arylsulfatase A9009
euchromatic histone-lysine N-methyltransferase 2160016
Bloom syndrome protein isoform 19009
D(1A) dopamine receptor3003
thyroid hormone receptor beta isoform a5005
thyroid hormone receptor beta isoform 22002
heat shock protein beta-11001
flap endonuclease 16006
geminin110011
peripheral myelin protein 224004
survival motor neuron protein isoform d1001
muscarinic acetylcholine receptor M15005
Histone deacetylase 30404
Solute carrier family 22 member 60101
Histone deacetylase 40404
Guanine nucleotide-binding protein G2002
Histone deacetylase 10404
Histone deacetylase 70404
Histone deacetylase 20404
Polyamine deacetylase HDAC100404
Histone deacetylase 11 0404
Ataxin-22002
Histone deacetylase 80404
Histone deacetylase 60404
Histone deacetylase 90404
Histone deacetylase 50404
ATP-dependent phosphofructokinase6006
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE7007
Chain A, TYROSYL-DNA PHOSPHODIESTERASE3003
Chain A, Ferritin light chain3003
aldehyde dehydrogenase 1 family, member A14004
cytochrome P450 2C19 precursor4004
lethal factor (plasmid)2002
lamin isoform A-delta109009
Metabotropic glutamate receptor 60135
Excitatory amino acid transporter 40202
Glutamate transporter homolog0011
Carbonic anhydrase 105010
Carbonic anhydrase 205212
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase0101
5-hydroxytryptamine receptor 1D0001
Glutamate receptor ionotropic, NMDA 1 0528
Excitatory amino acid transporter 10304
Excitatory amino acid transporter 20304
Excitatory amino acid transporter 30304
Glutamate receptor ionotropic, NMDA 2A 0528
Glutamate receptor ionotropic, NMDA 2B0629
Glutamate receptor ionotropic, NMDA 2C0528
Metabotropic glutamate receptor 10124
Metabotropic glutamate receptor 20124
Carbonic anhydrase-like protein, putative0003
Glutamate receptor ionotropic, NMDA 2D0528
Glutamate receptor ionotropic, NMDA 3B0528
Carbonic anhydrase 40203
Glutamate receptor ionotropic, NMDA 3A0528
peroxisome proliferator-activated receptor delta1001
Chain A, Acetylcholinesterase0011
Chain A, Acetylcholinesterase0011
Chain A, Acetylcholinesterase0011
Chain A, Acetylcholinesterase0011
Chain A, Acetylcholinesterase0011
Chain A, Acetylcholinesterase0011
Chain A, Acetylcholinesterase0011
Chain A, Putative Glycine Betaine-binding Abc Transporter Protein0011
Chain A, PUTATIVE GLYCINE BETAINE-BINDING ABC TRANSPORTER PROTEIN0011
Chain A, Glycine betaine/carnitine/choline-binding protein0011
Chain A, Glycine betaine/carnitine/choline-binding protein0011
Chain A, Glycine betaine/carnitine/choline-binding protein0011
Chain A, Glycine betaine/carnitine/choline-binding protein0011
Chain A, Choline-binding protein0011
Solute carrier family 22 member 10102
Solute carrier family 22 member 20001
Solute carrier family 22 member 1 0001
Solute carrier family 22 member 20101
Neuronal acetylcholine receptor subunit alpha-30101
Neuronal acetylcholine receptor subunit alpha-40202
Neuronal acetylcholine receptor subunit alpha-20101
Neuronal acetylcholine receptor subunit beta-20202
Neuronal acetylcholine receptor subunit beta-30101
Neuronal acetylcholine receptor subunit beta-40101
Neuronal acetylcholine receptor subunit alpha-50101
Acetylcholinesterase0303
Sodium- and chloride-dependent creatine transporter 10101
Choline O-acetyltransferase 0001
Neuronal acetylcholine receptor subunit alpha-60101
Neuronal acetylcholine receptor subunit alpha-90101
Neuronal acetylcholine receptor subunit alpha-70202
Solute carrier family 22 member 10102
High affinity choline transporter 10101
Neuronal acetylcholine receptor subunit alpha-100101
Solute carrier family 22 member 20102
Gamma-aminobutyric acid receptor subunit pi0404
Gamma-aminobutyric acid receptor subunit pi2406
Gamma-aminobutyric acid receptor subunit delta0404
Gamma-aminobutyric acid receptor subunit alpha-10101
Gamma-aminobutyric acid receptor subunit beta-10101
Gamma-aminobutyric acid receptor subunit alpha-20101
Gamma-aminobutyric acid receptor subunit alpha-30101
Gamma-aminobutyric acid receptor subunit alpha-10415
Gamma-aminobutyric acid receptor subunit beta-12406
Gamma-aminobutyric acid receptor subunit beta-10415
Gamma-aminobutyric acid receptor subunit delta2406
Gamma-aminobutyric acid receptor subunit gamma-20415
Gamma-aminobutyric acid receptor subunit gamma-22417
Gamma-aminobutyric acid receptor subunit alpha-52406
Gamma-aminobutyric acid receptor subunit alpha-32406
Gamma-aminobutyric acid receptor subunit alpha-40101
Gamma-aminobutyric acid receptor subunit gamma-20101
Gamma-aminobutyric acid receptor subunit gamma-12406
Gamma-aminobutyric acid receptor subunit alpha-22406
Gamma-aminobutyric acid receptor subunit alpha-42406
Gamma-aminobutyric acid receptor subunit beta-30415
Gamma-aminobutyric acid receptor subunit gamma-32406
Gamma-aminobutyric acid receptor subunit alpha-62406
Translocator protein0101
Translocator protein0101
Cholecystokinin receptor type A0101
Gastrin/cholecystokinin type B receptor0101
Gamma-aminobutyric acid receptor subunit alpha-50415
Gamma-aminobutyric acid receptor subunit alpha-30415
Gamma-aminobutyric acid receptor subunit alpha-20415
Gamma-aminobutyric acid receptor subunit beta-20415
Gamma-aminobutyric acid receptor subunit alpha-40415
Gamma-aminobutyric acid receptor subunit alpha-12417
Gamma-aminobutyric acid receptor subunit beta-32406
Gamma-aminobutyric acid receptor subunit beta-22417
Gamma-aminobutyric acid receptor subunit epsilon0404
Gamma-aminobutyric acid receptor subunit alpha-60415
Gamma-aminobutyric acid receptor subunit gamma-10404
GABA theta subunit2406
Gamma-aminobutyric acid receptor subunit gamma-30404
Gamma-aminobutyric acid receptor subunit epsilon2406
Gamma-aminobutyric acid receptor subunit theta0404
Sodium- and chloride-dependent creatine transporter 10101
Proton-coupled amino acid transporter 10202
estrogen-related nuclear receptor alpha6006
Sterol O-acyltransferase 20101
Sterol O-acyltransferase 10101
Retinal dehydrogenase 1 0101
Gamma-aminobutyric acid 0112
Gamma-aminobutyric acid receptor subunit rho-30011
thioredoxin reductase8008
ATAD5 protein, partial2002
Hsf1 protein0023
thyroid stimulating hormone receptor5005
bromodomain adjacent to zinc finger domain 2B2002
transcriptional regulator ERG isoform 33003
histone-lysine N-methyltransferase 2A isoform 2 precursor3003
histone acetyltransferase KAT2A isoform 14004
neuropeptide S receptor isoform A2002
Gamma-aminobutyric acid type B receptor subunit 20011
Gamma-aminobutyric acid type B receptor subunit 20101
Gamma-aminobutyric acid receptor subunit alpha-60112
Gamma-aminobutyric acid receptor subunit gamma-20112
Gamma-aminobutyric acid receptor subunit delta0112
Sodium- and chloride-dependent GABA transporter 10101
Gamma-aminobutyric acid receptor subunit rho-10022
Gamma-aminobutyric acid receptor subunit alpha-20112
Gamma-aminobutyric acid receptor subunit alpha-30112
Gamma-aminobutyric acid receptor subunit gamma-30112
Gamma-aminobutyric acid receptor subunit rho-20011
Sodium- and chloride-dependent GABA transporter 10101
Sodium- and chloride-dependent taurine transporter0101
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent GABA transporter 10101
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent betaine transporter0101
Sodium- and chloride-dependent betaine transporter0101
Sodium- and chloride-dependent GABA transporter 30101
Gamma-aminobutyric acid receptor subunit beta-10112
Gamma-aminobutyric acid receptor subunit alpha-10112
Gamma-aminobutyric acid receptor subunit beta-30112
4-aminobutyrate aminotransferase, mitochondrial0001
4-aminobutyrate aminotransferase, mitochondrial0001
TAR DNA-binding protein 432002
Sterol O-acyltransferase 10011
Gamma-aminobutyric acid receptor subunit alpha-50112
Gamma-aminobutyric acid receptor subunit pi0112
Gamma-aminobutyric acid receptor subunit alpha-40112
Platelet glycoprotein VI0011
Gamma-aminobutyric acid receptor subunit theta0112
Sodium- and chloride-dependent GABA transporter 20101
Gamma-aminobutyric acid receptor subunit gamma-10112
Gamma-aminobutyric acid type B receptor subunit 10011
Gamma-aminobutyric acid type B receptor subunit 10101
Chain A, GLUTAMATE RECEPTOR SUBUNIT 20101
Chain A, Glutamate Receptor Subunit 20101
Chain B, Glutamate Receptor Subunit 20101
Chain A, Slr1257 protein0011
Chain A, Glucosamine--fructose-6-phosphate aminotransferase [isomerizing]0101
glp-1 receptor, partial1001
GLS protein8008
TDP1 protein5005
beta-2 adrenergic receptor1001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 12002
Metabotropic glutamate receptor 80202
Glutamate receptor ionotropic, NMDA 2D0101
Glutamate receptor ionotropic, NMDA 3B0101
Glutathione reductase, mitochondrial0202
Bifunctional aspartokinase/homoserine dehydrogenase 10101
Prothrombin0011
Integrin beta-30011
Integrin alpha-IIb0011
ATP-citrate synthase 0101
Glutamate receptor 10426
Glutamate receptor 20426
Glutamate receptor 30426
Glutamate receptor 40426
Glutamate receptor ionotropic, kainate 10426
Metabotropic glutamate receptor 10112
Metabotropic glutamate receptor 20011
Metabotropic glutamate receptor 30011
Metabotropic glutamate receptor 40112
Metabotropic glutamate receptor 50011
Metabotropic glutamate receptor 60011
Metabotropic glutamate receptor 70011
Glutamate receptor ionotropic, kainate 10325
Metabotropic glutamate receptor 50124
Glutamate receptor ionotropic, kainate 20437
Glutamate receptor 10426
Glutamate receptor 20325
Glutamate receptor 30123
Glutamate receptor ionotropic, kainate 30404
Metabotropic glutamate receptor 80011
Glutamate receptor 40314
Excitatory amino acid transporter 3 0001
Glutamate racemase0001
Metabotropic glutamate receptor 80112
Fatty-acid amide hydrolase 10101
Glutamate receptor ionotropic, kainate 40404
Glutamate carboxypeptidase 20101
Glutamate receptor ionotropic, NMDA 10101
Glutamate receptor ionotropic, NMDA 2A0101
Glutamate receptor ionotropic, kainate 20325
Glutamate receptor ionotropic, kainate 30303
Glutamate receptor ionotropic, NMDA 2B0101
Metabotropic glutamate receptor 70113
Metabotropic glutamate receptor 30113
Metabotropic glutamate receptor 40113
Glutamate receptor ionotropic, NMDA 2C0101
Glutamate receptor ionotropic, kainate 50303
Glutamate receptor ionotropic, kainate 50404
Glutamate racemase0001
Prolyl 4-hydroxylase0101
Glutamate receptor ionotropic, NMDA 3A0101
Alpha-ketoglutarate-dependent dioxygenase FTO0101
Chain A, Membrane lipoprotein tmpC0011
Chain A, Membrane lipoprotein tmpC0011
Chain A, Membrane lipoprotein tmpC0011
Purine nucleoside phosphorylase0001
Adenosylhomocysteinase0102
endonuclease IV3003
Fumarate hydratase1001
Smad32002
hypothetical protein, conserved1001
polyprotein1001
alpha-galactosidase1001
IDH12002
peripheral myelin protein 22 isoform 14004
cytochrome P450 2C9 precursor5005
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 13003
vitamin D3 receptor isoform VDRA2002
chromobox protein homolog 17007
urokinase-type plasminogen activator precursor1001
plasminogen precursor1001
urokinase plasminogen activator surface receptor precursor1001
M-phase phosphoprotein 83003
Histamine H2 receptor5005
Uracil nucleotide/cysteinyl leukotriene receptor0202
Chain A, Glutamate receptor 20011
Chain A, Glutamate receptor 20011
Chain C, Glutamate receptor 20011
importin subunit beta-1 isoform 11001
snurportin-11001
nuclear receptor ROR-gamma isoform 14004
DNA polymerase kappa isoform 13003
Cellular tumor antigen p535005
Glutamate receptor ionotropic, kainate 40101
Chain A, Putative fructose-1,6-bisphosphate aldolase3003
GALC protein2002
EWS/FLI fusion protein3003
cytochrome P450 2D64004
cytochrome P450 2D6 isoform 14004
nuclear factor erythroid 2-related factor 2 isoform 23003
potassium voltage-gated channel subfamily H member 2 isoform d3003
mitogen-activated protein kinase 12002
Bile salt export pump010010
Sodium channel protein type 1 subunit alpha0101
Sodium channel protein type 2 subunit alpha0303
Sodium channel protein type 3 subunit alpha0101
Dihydrofolate reductase0202
Potassium voltage-gated channel subfamily E member 10101
Sodium channel protein type 4 subunit alpha0204
Potassium voltage-gated channel subfamily KQT member 10101
Potassium voltage-gated channel subfamily H member 20303
Voltage-dependent L-type calcium channel subunit alpha-1C0202
Sodium channel protein type 5 subunit alpha0202
Sodium channel protein type 9 subunit alpha0202
Sodium channel protein type 2 subunit alpha0303
Sodium channel protein type 3 subunit alpha0303
Sigma non-opioid intracellular receptor 10101
Sodium channel protein type 10 subunit alpha0101
chaperonin-containing TCP-1 beta subunit homolog1001
ATP-binding cassette sub-family C member 30606
Multidrug resistance-associated protein 40606
Synaptic vesicle glycoprotein 2A0303
Canalicular multispecific organic anion transporter 10505
Type-1A angiotensin II receptor 0101
Type-1B angiotensin II receptor0202
Type-2 angiotensin II receptor0101
cGMP-inhibited 3',5'-cyclic phosphodiesterase B0101
cGMP-inhibited 3',5'-cyclic phosphodiesterase A0101
5-hydroxytryptamine receptor 1A0101
Type-1 angiotensin II receptor0101
cytochrome P450 family 3 subfamily A polypeptide 42002
67.9K protein1001
Solute carrier family 22 member 200101
Solute carrier family 22 member 60101
Rap guanine nucleotide exchange factor 41001
G2002
Interferon beta3003
HLA class I histocompatibility antigen, B alpha chain 2002
Replicase polyprotein 1ab0101
Inositol hexakisphosphate kinase 12002
cytochrome P450 2C9, partial2002
Sodium channel protein type 1 subunit alpha0202
GLI family zinc finger 33003
nuclear receptor subfamily 1, group I, member 33003
pregnane X nuclear receptor3003
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a2002
atrial natriuretic peptide receptor 1 precursor1001
parathyroid hormone/parathyroid hormone-related peptide receptor precursor1001
nuclear factor erythroid 2-related factor 2 isoform 12002
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Voltage-dependent L-type calcium channel subunit alpha-1F0101
Dihydrofolate reductase 0101
Cytochrome P450 2C9 0101
Aldo-keto reductase family 1 member B10101
5-hydroxytryptamine receptor 1A0101
Inositol monophosphatase 12002
Voltage-dependent L-type calcium channel subunit alpha-1D 0101
Voltage-dependent L-type calcium channel subunit alpha-1S0101
Chain A, Beta-lactamase1001
peroxisome proliferator activated receptor gamma3003
Spike glycoprotein1001
Carbonic anhydrase 40407
Carbonic anhydrase 5A, mitochondrial0429
Taste receptor type 2 member 310011
Taste receptor type 2 member 600011
Carbonic anhydrase 90407
Sodium/potassium/calcium exchanger 40112
Taste receptor type 2 member 140011
Taste receptor type 2 member 130011
Taste receptor type 2 member 90011
Taste receptor type 2 member 40011
Sodium/potassium/calcium exchanger 20112
thyroid stimulating hormone receptor2002
DNA polymerase beta1001
DNA polymerase iota isoform a (long)2002
Rap guanine nucleotide exchange factor 31001
runt-related transcription factor 1 isoform AML1b1001
core-binding factor subunit beta isoform 21001
cytochrome P450 3A4 isoform 12002
caspase-1 isoform alpha precursor1001
Cholinesterase0303
Acetylcholinesterase0101
Acetylcholinesterase 0101
Caspase-71001
Chain A, HADH2 protein1001
Chain B, HADH2 protein1001
Luciferase2002
D(2) dopamine receptor0314
D(3) dopamine receptor0202
D(4) dopamine receptor0112
D(3) dopamine receptor0213
D(2) dopamine receptor0101
D(2) dopamine receptor0505
5-hydroxytryptamine receptor 1A0101
D(2) dopamine receptor0101
D(1A) dopamine receptor0011
D(1B) dopamine receptor0011
5-hydroxytryptamine receptor 2A0011
Amyloid-beta precursor protein0101
Chain A, Carbonic anhydrase 10101
Carbonic anhydrase 0002
Carbonic anhydrase 0202
Carbonic anhydrase 0202
Carbonic anhydrase 0202
Carbonic anhydrase0202
Carbonic anhydrase0202
Carbonic anhydrase 120306
Prolyl endopeptidase0202
Carbonic anhydrase-related protein 110001
Carbonic anhydrase 30304
Cytochrome P450 3A40101
Polyunsaturated fatty acid 5-lipoxygenase0202
Cytochrome P450 2C80101
Arachidonate 5-lipoxygenase-activating protein0202
Carbonic anhydrase 60306
Carbonic anhydrase 5A, mitochondrial0101
Carbonic anhydrase 20101
Delta-type opioid receptor0202
Cytochrome P450 2C190101
Delta-type opioid receptor0202
Mu-type opioid receptor0202
Carbonic anhydrase0102
Carbonic anhydrase 70306
Carbonic anhydrase0203
Carbonic anhydrase 0202
Mu-type opioid receptor0202
Beta-carbonic anhydrase 10202
Carbonic anhydrase 20202
Carbonic anhydrase, alpha family 0202
Carbonic anhydrase 0203
Carbonic anhydrase0102
Carbonic anhydrase0203
Carbonic anhydrase 0202
Delta carbonic anhydrase0202
Carbonic anhydrase 0203
Carbonic anhydrase 130303
Carbonic anhydrase 150203
Carbonic anhydrase 130104
Carbonic anhydrase 140306
Carbonic anhydrase 5B, mitochondrial0306
Chain A, Trp Rna-binding Attenuation Protein0011
Chain K, Trp Rna-binding Attenuation Protein0011
Chain B, tryptophanyl-tRNA synthetase0011
Chain C, Tryptophanyl-tRNA synthetase II0011
thioredoxin glutathione reductase1001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)1001
aryl hydrocarbon receptor1001
Dihydrofolate reductase0202
Plasma kallikrein0001
Myeloperoxidase0101
Alkaline phosphatase, tissue-nonspecific isozyme0101
Stromelysin-10101
Intestinal-type alkaline phosphatase0101
Indoleamine 2,3-dioxygenase 10102
Substance-P receptor0101
Tryptophan 2,3-dioxygenase0102
Large neutral amino acids transporter small subunit 10101
SLC16A10 protein0001
Tryprostatin B synthase0001
2-C-methyl-D-erythritol 2,4-cyclodiphosphate synthase0011
Monocarboxylate transporter 100001
Phospholipase A-2-activating protein0101
Cocaine esterase0101
UDP-glucuronosyltransferase 1A90001
Bile salt export pump0101
Aldo-keto reductase family 1 member B10101
Cytochrome P450 2A60101
Tissue factor0101
Aldo-keto reductase family 1 member A10101
UDP-glucuronosyltransferase 2B70102
UDP-glucuronosyltransferase 1-60001
Liver carboxylesterase 10101
Nuclear receptor ROR-gamma1001
Aldo-keto reductase family 1 member A10101
Aldo-keto reductase family 1 member A10101
Aldo-keto reductase family 1 member B70101
Sigma non-opioid intracellular receptor 10101
ATPase family AAA domain-containing protein 51001
NAD-dependent protein deacylase sirtuin-5, mitochondrial0101
Carbonic anhydrase 20101
Translocator protein0101
Amine oxidase [flavin-containing] B0101
Sodium-dependent dopamine transporter0101
Amine oxidase [flavin-containing] B0101
Carbonic anhydrase 30101
Amine oxidase [flavin-containing]0101
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]