Page last updated: 2024-09-27

Familial Fatal Insomnia

An autosomal dominant disorder characterized by degeneration of the THALAMUS and progressive insomnia. It is caused by a mutation in the prion protein (PRIONS).

Synonyms(4)

Synonym
Familial Fatal Insomnia
Fatal Familial Insomnia
Insomnia, Fatal Familial
Insomnia Familial Fatal

Research Excerpts

Research

Studies (32)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's15 (46.88)18.1394
2010's15 (46.88)28.8240
2020's2 (6.25)9.53
DrugIndicatedRelationship StrengthStudiesTrials
quinacrine0low10
melatonin0low10
amantadine0low10
chlorpromazine0low20
aspartic acid0low20
leucine0low10
methionine0low90
asparagine0low20
valine0low40
pyrazines0low10
4-methoxyamphetamine0low10
fluorodeoxyglucose f180low70
s200980low10
bortezomib0low10
micafungin0low10
amphotericin b0low10
casein kinase ii0low10
alpha-synuclein0low10
calpain0low10

Protein Targets (464)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
5-hydroxytryptamine receptor 2C0213
5-hydroxytryptamine receptor 2A0213
5-hydroxytryptamine receptor 1A0314
5-hydroxytryptamine receptor 1B0314
5-hydroxytryptamine receptor 1D0112
5-hydroxytryptamine receptor 1F0112
5-hydroxytryptamine receptor 2B0213
5-hydroxytryptamine receptor 60011
Sodium-dependent serotonin transporter0213
Sodium-dependent serotonin transporter0101
5-hydroxytryptamine receptor 7 0112
5-hydroxytryptamine receptor 5A0011
5-hydroxytryptamine receptor 5B0011
5-hydroxytryptamine receptor 3A0011
5-hydroxytryptamine receptor 4 0011
5-hydroxytryptamine receptor 3B0011
acetylcholinesterase2002
thioredoxin reductase5005
RAR-related orphan receptor gamma5005
NFKB1 protein, partial3003
GLI family zinc finger 33003
AR protein4004
signal transducer and activator of transcription 6, interleukin-4 induced1001
thyroid stimulating hormone receptor1001
regulator of G-protein signaling 43003
EWS/FLI fusion protein4004
estrogen nuclear receptor alpha4004
putative alpha-glucosidase1001
euchromatic histone-lysine N-methyltransferase 26006
thyroid hormone receptor beta isoform 23003
heat shock protein beta-13003
peripheral myelin protein 223003
histone acetyltransferase KAT2A isoform 13003
lamin isoform A-delta106006
Solute carrier family 22 member 20102
Solute carrier family 22 member 1 0102
Glutamate receptor ionotropic, NMDA 2D0202
Glutamate receptor ionotropic, NMDA 3B0202
Matrix protein 20112
Matrix protein 20123
Glutamate receptor ionotropic, NMDA 1 0303
Spike glycoprotein2114
Glutamate receptor ionotropic, NMDA 2A 0303
Glutamate receptor ionotropic, NMDA 2B0303
Glutamate receptor ionotropic, NMDA 2C0303
Glutamate receptor ionotropic, NMDA 10202
Glutamate receptor ionotropic, NMDA 2A0202
Glutamate receptor ionotropic, NMDA 2B0202
Glutamate receptor ionotropic, NMDA 2C0202
Glutamate receptor ionotropic, NMDA 2D0303
Solute carrier family 22 member 10101
Glutamate receptor ionotropic, NMDA 3A0202
Glutamate receptor ionotropic, NMDA 3B0303
Multidrug and toxin extrusion protein 10101
Solute carrier family 22 member 20101
Glutamate receptor ionotropic, NMDA 3A0303
ATP-dependent phosphofructokinase2002
Chain A, HADH2 protein2002
Chain B, HADH2 protein2002
Chain A, Cruzipain1001
interleukin 82002
phosphopantetheinyl transferase4004
GLS protein1001
glucocorticoid receptor [Homo sapiens]3003
farnesoid X nuclear receptor3003
peroxisome proliferator-activated receptor delta3003
vitamin D (1,25- dihydroxyvitamin D3) receptor3003
tumor necrosis factor1001
vitamin D3 receptor isoform VDRA2002
nuclear factor erythroid 2-related factor 2 isoform 13003
Cytochrome P450 1A20213
Cytochrome P450 3A40101
Cytochrome P450 2D60314
Cytochrome P450 2C9 0202
Cytochrome P450 2C190101
Nuclear receptor ROR-gamma2002
Potassium voltage-gated channel subfamily H member 20303
Aurora kinase B0101
Broad substrate specificity ATP-binding cassette transporter ABCG20202
Carbonic anhydrase 10103
Carbonic anhydrase 20103
Neutral amino acid transporter A0101
Neutral amino acid transporter B(0)0101
Carbonic anhydrase-like protein, putative0002
Amino acid transporter0101
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE1001
Chain A, TYROSYL-DNA PHOSPHODIESTERASE3003
Chain A, Ferritin light chain2002
aldehyde dehydrogenase 1 family, member A12002
arylsulfatase A3003
Bloom syndrome protein isoform 13003
cytochrome P450 2C19 precursor1001
lethal factor (plasmid)2002
Metabotropic glutamate receptor 60011
Excitatory amino acid transporter 40101
Glutamate transporter homolog0011
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase0101
5-hydroxytryptamine receptor 1D0001
Excitatory amino acid transporter 10101
Excitatory amino acid transporter 20101
Excitatory amino acid transporter 30101
Metabotropic glutamate receptor 10011
Metabotropic glutamate receptor 20011
Carbonic anhydrase 40001
Chain H, Proteasome component PUP10101
Chain I, Proteasome component PUP30101
Chain K, Proteasome component PRE20101
Chain L, Proteasome component C50101
Chain K, Proteasome component PRE20101
Chain L, Proteasome component C50101
Proteasome subunit beta type-110102
Fumarate hydratase3003
PPM1D protein1001
TDP1 protein3003
caspase 7, apoptosis-related cysteine protease3003
estrogen receptor 2 (ER beta)3003
nuclear receptor subfamily 1, group I, member 34004
progesterone receptor2002
cytochrome P450 family 3 subfamily A polypeptide 43003
retinoic acid nuclear receptor alpha variant 13003
retinoid X nuclear receptor alpha2002
estrogen-related nuclear receptor alpha4004
pregnane X nuclear receptor3003
polyprotein3003
peroxisome proliferator activated receptor gamma2002
caspase-33003
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a2002
nuclear receptor subfamily 1, group I, member 21001
Histone H2A.x2002
Caspase-72002
caspase-32002
26S proteasome non-ATPase regulatory subunit 110101
26S proteasome non-ATPase regulatory subunit 120101
26S proteasome non-ATPase regulatory subunit 140101
Proteasome subunit alpha type-70102
Histone deacetylase 30101
ATP-binding cassette sub-family C member 30404
Multidrug resistance-associated protein 40404
26S proteasome non-ATPase regulatory subunit 30101
Carbonic anhydrase 120101
Voltage-dependent calcium channel gamma-2 subunit1001
Bile salt export pump0505
Prothrombin0101
Chymotrypsinogen A0101
Interferon beta3003
Cellular tumor antigen p533003
Carbonic anhydrase 30101
Cathepsin B0101
Neutrophil elastase0101
Cathepsin G0202
Lysosomal protective protein0101
Chymotrypsinogen B0101
26S proteasome regulatory subunit 6A0101
Glutamate receptor 21001
Nuclear factor NF-kappa-B p105 subunit0101
Proteasome subunit beta type-10103
Carbonic anhydrase 40101
Carbonic anhydrase 60101
Chymase0101
Proteasome subunit alpha type-10102
Proteasome subunit alpha type-20102
Proteasome subunit alpha type-30102
Proteasome subunit alpha type-40102
Proteasome subunit beta type-80102
Proteasome subunit beta type-80101
Proteasome subunit beta type-90102
Proteasome subunit alpha type-50102
Proteasome subunit beta type-40102
Proteasome subunit beta type-60102
Proteasome subunit beta type-50204
Carbonic anhydrase 5A, mitochondrial0101
26S proteasome regulatory subunit 70101
Lon protease homolog, mitochondrial0101
Proteasome subunit beta type-100102
Carbonic anhydrase 70101
26S proteasome regulatory subunit 6B0101
26S proteasome non-ATPase regulatory subunit 80101
Proteasome subunit beta type-30102
Proteasome subunit beta type-20103
26S proteasome non-ATPase regulatory subunit 70101
26S proteasome non-ATPase regulatory subunit 40101
26S proteasome complex subunit SEM10101
Proteasome subunit alpha type-60113
26S proteasome regulatory subunit 40101
26S proteasome regulatory subunit 80101
26S proteasome regulatory subunit 10B0101
Beta-carbonic anhydrase 10101
Nuclear factor NF-kappa-B p100 subunit 0101
Transcription factor p650101
26S proteasome non-ATPase regulatory subunit 20101
Histone deacetylase 10101
26S proteasome non-ATPase regulatory subunit 60101
Proteasomal ubiquitin receptor ADRM10101
Carbonic anhydrase 90101
ATP-dependent Clp protease proteolytic subunit0112
Carbonic anhydrase 130101
NACHT, LRR and PYD domains-containing protein 3 0101
Proteasome subunit alpha-type 80102
Histone deacetylase 20101
Canalicular multispecific organic anion transporter 10404
ATPase family AAA domain-containing protein 51001
Proteasome subunit beta type-70102
26S proteasome non-ATPase regulatory subunit 10101
Ataxin-23003
Carbonic anhydrase 150101
Histone deacetylase 80101
Histone deacetylase 60101
Carbonic anhydrase 140101
26S proteasome non-ATPase regulatory subunit 130101
Carbonic anhydrase 5B, mitochondrial0101
Chain A, Protein kinase CK2, alpha Subunit0101
Chain A, PROTEIN KINASE CK2, alpha SUBUNIT0101
Chain A, Protein Kinase Ck2, Alpha Subunit0101
endonuclease IV1001
dopamine D1 receptor2002
ATAD5 protein, partial4004
Thrombopoietin2002
Parkin1001
NPC intracellular cholesterol transporter 1 precursor2002
peripheral myelin protein 22 isoform 12002
mitogen-activated protein kinase 12002
ras-related protein Rab-9A4004
serine/threonine-protein kinase mTOR isoform 13003
DNA polymerase iota isoform a (long)2002
geminin3003
cytochrome P450 3A4 isoform 13003
Serine/threonine-protein kinase Sgk10101
Gamma-aminobutyric acid receptor subunit pi3003
Gamma-aminobutyric acid receptor subunit beta-13003
Ribosyldihydronicotinamide dehydrogenase [quinone]0314
Gamma-aminobutyric acid receptor subunit delta3003
Gamma-aminobutyric acid receptor subunit gamma-23003
Casein kinase II subunit alpha 0101
Casein kinase II subunit alpha'0202
Gamma-aminobutyric acid receptor subunit alpha-53003
Gamma-aminobutyric acid receptor subunit alpha-33003
Cyclin-A20101
Gamma-aminobutyric acid receptor subunit gamma-13003
Gamma-aminobutyric acid receptor subunit alpha-23003
Cyclin-dependent kinase 20101
Gamma-aminobutyric acid receptor subunit alpha-43003
Gamma-aminobutyric acid receptor subunit gamma-33003
Gamma-aminobutyric acid receptor subunit alpha-63003
D(1A) dopamine receptor3003
Gamma-aminobutyric acid receptor subunit alpha-13003
Gamma-aminobutyric acid receptor subunit beta-33003
Gamma-aminobutyric acid receptor subunit beta-23003
Casein kinase II subunit beta0202
Casein kinase II subunit beta0101
Casein kinase II subunit alpha0202
Cyclin-A10101
Dual specificity mitogen-activated protein kinase kinase 10101
Dual specificity tyrosine-phosphorylation-regulated kinase 1A0101
Ribosomal protein S6 kinase alpha-10101
Casein kinase II subunit alpha 30202
GABA theta subunit3003
Gamma-aminobutyric acid receptor subunit epsilon3003
Single-stranded DNA cytosine deaminase1001
USP1 protein, partial2002
Microtubule-associated protein tau1001
Smad31001
NADPH oxidase 10101
G2002
cytochrome P450 2D62002
67.9K protein2002
Snq2p0001
IDH12002
Adenylate cyclase type 1 0202
thyroid stimulating hormone receptor1001
v-jun sarcoma virus 17 oncogene homolog (avian)1001
Spike glycoprotein0112
Replicase polyprotein 1ab0112
cytochrome P450 2D6 isoform 12002
cellular tumor antigen p53 isoform a1001
D(1A) dopamine receptor2002
chromobox protein homolog 13003
potassium voltage-gated channel subfamily H member 2 isoform d1001
histone deacetylase 9 isoform 31001
atrial natriuretic peptide receptor 2 precursor1001
DNA polymerase kappa isoform 12002
survival motor neuron protein isoform d1001
M-phase phosphoprotein 83003
muscarinic acetylcholine receptor M12002
Polyunsaturated fatty acid lipoxygenase ALOX15B1001
Transmembrane protease serine 20112
Transient receptor potential cation channel subfamily V member 10101
Voltage-dependent L-type calcium channel subunit alpha-1F0101
Bile salt export pump0101
5-hydroxytryptamine receptor 40202
Potassium channel subfamily K member 20101
Epidermal growth factor receptor0101
HLA class I histocompatibility antigen, B alpha chain 2002
Albumin0011
Albumin0011
Major prion protein0022
Sarcoplasmic/endoplasmic reticulum calcium ATPase 10101
Receptor tyrosine-protein kinase erbB-20101
Major prion protein0112
Integrin beta-31001
Tyrosine-protein kinase Fyn0101
Procathepsin L0112
Aldo-keto reductase family 1 member B10404
Muscarinic acetylcholine receptor M20404
Muscarinic acetylcholine receptor M40404
ATP-dependent translocase ABCB10113
Muscarinic acetylcholine receptor M10202
Muscarinic acetylcholine receptor M30202
Muscarinic acetylcholine receptor M40202
Integrin alpha-IIb1001
5-hydroxytryptamine receptor 1A0101
Muscarinic acetylcholine receptor M50202
Muscarinic acetylcholine receptor M50404
Alpha-2A adrenergic receptor0404
Replicase polyprotein 1a0112
Replicase polyprotein 1ab0112
Replicase polyprotein 1ab0112
Cys-loop ligand-gated ion channel0101
Calmodulin-10213
Replicase polyprotein 1ab0213
Muscarinic acetylcholine receptor M20202
Muscarinic acetylcholine receptor M10404
Polyunsaturated fatty acid lipoxygenase ALOX150101
Angiotensin-converting enzyme0202
D(2) dopamine receptor0203
Alpha-1B adrenergic receptor0202
Sphingomyelin phosphodiesterase0101
Alpha-2B adrenergic receptor0404
Alpha-2C adrenergic receptor0404
D0202
D(3) dopamine receptor0202
Alpha-2B adrenergic receptor0202
D(2) dopamine receptor0101
Muscarinic acetylcholine receptor M30404
Substance-K receptor0202
D(1A) dopamine receptor0203
D(4) dopamine receptor0203
D(1B) dopamine receptor0102
Adenylate cyclase type 30202
Alpha-2C adrenergic receptor0202
UDP-glucuronosyltransferase 1A40001
Alpha-2A adrenergic receptor0202
Alpha-1D adrenergic receptor0202
Sodium-dependent noradrenaline transporter 0404
Sodium-dependent dopamine transporter0202
Histamine H2 receptor0404
Alpha-1D adrenergic receptor0404
D(1B) dopamine receptor0202
Adenylate cyclase type 20202
Adenylate cyclase type 40202
5-hydroxytryptamine receptor 2A0404
5-hydroxytryptamine receptor 2C0303
Trypanothione reductase0404
D(4) dopamine receptor0202
Histamine H1 receptor0101
Melanocortin receptor 40202
Melanocortin receptor 50202
Pleiotropic ABC efflux transporter of multiple drugs0102
5-hydroxytryptamine receptor 70101
Histamine H1 receptor0202
Mu-type opioid receptor0202
D(3) dopamine receptor0405
Sodium channel protein type 1 subunit alpha0101
Sodium channel protein type 4 subunit alpha0101
Adenylate cyclase type 80202
Delta-type opioid receptor0202
Kappa-type opioid receptor0202
5-hydroxytryptamine receptor 2B0202
Melanocortin receptor 30202
Alpha-1A adrenergic receptor0303
Histamine H2 receptor1001
5-hydroxytryptamine receptor 60202
5-hydroxytryptamine receptor 70101
Cytochrome P450 2J20101
Gastrin/cholecystokinin type B receptor0101
D(2) dopamine receptor0203
Calmodulin 0112
Aldehyde oxidase 10202
Sodium channel protein type 7 subunit alpha0101
Voltage-dependent L-type calcium channel subunit alpha-1D 0101
Sodium-dependent dopamine transporter 0202
Adenylate cyclase type 60202
Adenylate cyclase type 50202
Aldehyde oxidase0303
Voltage-dependent L-type calcium channel subunit alpha-1S0101
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Sodium channel protein type 5 subunit alpha0101
Sodium channel protein type 9 subunit alpha0101
Nuclear receptor subfamily 3 group C member 3 0202
Adenylyl cyclase 7 0202
Inositol hexakisphosphate kinase 12002
D0101
Sodium channel protein type 2 subunit alpha0101
Sigma non-opioid intracellular receptor 10202
Angiotensin-converting enzyme 2 0112
Sodium channel protein type 3 subunit alpha0101
Sodium channel protein type 11 subunit alpha0101
Sodium channel protein type 8 subunit alpha0101
Histamine H3 receptor0202
Sodium channel protein type 10 subunit alpha0101
cytochrome P450 2C9, partial2002
Chain A, AMINOPEPTIDASE0101
Chain A, AMINOPEPTIDASE0101
Chain A, Leucine Aminopeptidase0101
Alkaline phosphatase, tissue-nonspecific isozyme0101
Intestinal-type alkaline phosphatase0101
Large neutral amino acids transporter small subunit 10303
Proton-coupled amino acid transporter 10102
Phospholipase A-2-activating protein0101
Chain A, 2-oxoglutarate Oxygenase1001
ClpP1001
aryl hydrocarbon receptor1001
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 11001
Cytochrome P450 1A10101
Cytochrome P450 1A2 0101
Myeloperoxidase0101
Cholinesterase0101
Cytochrome P450 2C11 0101
Aromatase0101
Acetylcholinesterase0202
Metabotropic glutamate receptor 10101
Acetylcholinesterase 0101
Mu-type opioid receptor0101
Nociceptin receptor0101
Melatonin receptor type 1A0426
Melatonin receptor type 1C0011
Melatonin receptor type 1A0224
Melatonin receptor type 1B0426
Melatonin receptor type 1C0224
Melatonin receptor type 1B0224
Nuclear factor erythroid 2-related factor 20001
Cytochrome P450 1B10101
Palmitoleoyl-protein carboxylesterase NOTUM0101
Carboxylic ester hydrolase 0101
Chain A, Methionyl-tRNA synthetase0011
Chain A, Methionyl-tRNA synthetase0011
Chain A, Aminopeptidase0101
Chain A, Methionine aminopeptidase0101
S-ribosylhomocysteine lyase0101
Adenylate cyclase type 50001
S-adenosylmethionine synthase isoform type-10001
S-adenosylmethionine synthase isoform type-20001
Fatty acid synthase0101
N0101
NPYLR7B0011
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)1001
cystic fibrosis transmembrane conductance regulator0101
flap endonuclease 11001
ubiquitin carboxyl-terminal hydrolase 2 isoform a1001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 11001
tumor susceptibility gene 101 protein1001
Glutathione reductase, mitochondrial0101
Riboflavin-binding protein0112
Histidine-rich protein PFHRP-II0101
DNA-directed RNA polymerase subunit alpha0101
DNA-directed RNA polymerase subunit omega0101
DNA-directed RNA polymerase subunit beta'0101
DNA-directed RNA polymerase subunit beta0101
Phospholipase A2, membrane associated0101
Phospholipase A2, membrane associated0101
Acidic phospholipase A2 20101
Cholinesterase0101
Cyclic GMP-AMP synthase0101
2,3-bisphosphoglycerate-independent phosphoglycerate mutase1001
Chain A, Arginase 10101
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]