MCID: LMB024
MIFTS: 38

Limbic Encephalitis malady

Category: Rare diseases

Aliases & Classifications for Limbic Encephalitis

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Aliases & Descriptions for Limbic Encephalitis:

Name: Limbic Encephalitis 48 50 68

Classifications:



Summaries for Limbic Encephalitis

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NIH Rare Diseases:48 Limbic encephalitis represents a group of autoimmune conditions characterized by inflammation of the limbic system and other parts of the brain. the cardinal sign of limbic encephalitis is a severe impairment of short-term memory; however, symptoms may also include confusion, psychiatric symptoms, and seizures. the symptoms typically develop over a few weeks or months, but they may evolve over a few days. limbic encephalitis is often associated with an underlying neoplasm (paraneoplastic limbic encephalitis); however some cases never have a neoplasm identified (non-paraneoplastic limbic encephalitis). delayed diagnosis is common, but improvements are being made to assist in early detection. various tests including imaging studies (mri, pet) laboratory tests (csf analysis), and tests that measure the electrical activity of the brain (eeg) may be utilized to confirm a diagnosis. treatment includes removal of the neoplasm (if identified) and immunotherapy. last updated: 4/26/2016

MalaCards based summary: Limbic Encephalitis is related to non-herpetic acute limbic encephalitis and posttransplant acute limbic encephalitis. An important gene associated with Limbic Encephalitis is GAD1 (Glutamate Decarboxylase 1), and among its related pathways are LGI-ADAM interactions and Biogenic Amine Synthesis. Affiliated tissues include brain, testes and lung, and related mouse phenotypes are behavior/neurological and nervous system.

Wikipedia:71 Limbic encephalitis is a form of encephalitis, a disease characterised by inflammation of the brain.... more...

Related Diseases for Limbic Encephalitis

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Diseases related to Limbic Encephalitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 97)
idRelated DiseaseScoreTop Affiliating Genes
1non-herpetic acute limbic encephalitis12.1
2posttransplant acute limbic encephalitis12.1
3limbic encephalitis with dpp6 antibodies12.0
4classic paraneoplastic limbic encephalitis12.0
5limbic encephalitis with lgi1 antibodies12.0
6limbic encephalitis with ncmags antibodies12.0
7limbic encephalitis with nmda receptor antibodies12.0
8limbic encephalitis with caspr2 antibodies11.9
9encephalitis11.0
10neuropathy, hereditary sensory and autonomic, type ii11.0
11epilepsy10.2
12neuronitis10.2
13lung cancer10.2
14teratoma10.2
15thymoma10.2
16glioma10.1
17lymphoma10.1
18status epilepticus10.1
19relapsing polychondritis10.1
20cone dystrophy 410.1ADAM22, LGI1
21hemangioblastoma10.1GRIN2B, GSR
22triphalangeal thumbs and dislocation of patella10.1GAD1, GAD2
23temporal lobe epilepsy10.1
24atrial tachyarrhythmia with short pr interval10.0AMPH, GAD1, GAD2
25hodgkin lymphoma10.0
26dementia10.0
27deafness, autosomal recessive 2910.0GAD1, GAD2
28adenocarcinoma10.0
29testicular cancer10.0
30cerebellar degeneration10.0
31idiopathic generalized epilepsy9.9GRIA1, GRIN2B, LGI1
32breast cancer9.9
33prostatitis9.9
34neurosyphilis9.9
35paraneoplastic cerebellar degeneration9.9
36encephalopathy9.9
37paraneoplastic syndromes9.9
38prostate cancer9.8
39renal cell carcinoma9.8
40cerebellar ataxia9.8
41creutzfeldt-jakob disease9.8
42stiff-person syndrome9.8
43leukemia9.8
44myasthenia gravis9.8
45squamous cell carcinoma9.8
46ovarian cancer9.8
47psychotic disorder9.8
48cerebritis9.8
49anterograde amnesia9.8
50viral encephalitis9.8

Graphical network of the top 20 diseases related to Limbic Encephalitis:



Diseases related to limbic encephalitis

Symptoms & Phenotypes for Limbic Encephalitis

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MGI Mouse Phenotypes related to Limbic Encephalitis according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053867.6ADAM22, AMPH, BRSK2, ELAVL3, GAD1, GAD2
2MP:00036316.8ADAM22, AMPH, BRSK2, ELAVL3, GAD1, GAD2

Drugs & Therapeutics for Limbic Encephalitis

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Drugs for Limbic Encephalitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1ImmunoglobulinsPhase 26394
2AntibodiesPhase 26394
3Immunoglobulins, IntravenousPhase 2333
4Rho(D) Immune GlobulinPhase 2326
5gamma-GlobulinsPhase 2326
6AutoantibodiesPhase 2130
7
Prednisoneapproved, vet_approved139853-03-25865
Synonyms:
(1S,2R,10S,11S,14R,15S)-14-hydroxy-14-(2-hydroxyacetyl)-2,15-dimethyltetracyclo[8.7.0.0^{2,7}.0^{11,15}]heptadeca-3,6-diene-5,17-dione
(8S,9S,10R,13S,14S,17R)-17-hydroxy-17-(2-hydroxyacetyl)-10,13-dimethyl-6,7,8,9,12,14,15,16-octahydrocyclopenta[a]phenanthrene-3,11-dione
(8xi,9xi,14xi)-17,21-dihydroxypregna-1,4-diene-3,11,20-trione
.delta. E
.delta.(sup1)-Cortisone
.delta.-Cortelan
.delta.-Cortisone
.delta.-Cortone
.delta.-E
.delta.1-Cortisone
.delta.1-Dehydrocortisone
.delta.sone
1,2-Dehydrocortisone
1,4-Pregnadiene-17-alpha,21-diol-3,11,20-trione
1,4-Pregnadiene-17.alpha.,21-diol-3,11,20-trione
1,4-Pregnadiene-17alpha,21-diol-3,11,20-trione
1-Cortisone
1-Dehydrocortisone
17,21-Dihydroxypregna-1,4-diene-3,11,20-trione
17alpha,21-Dihydroxy-1,4-pregnadiene-3,11,20-trione
53-03-2
68-59-7
81552_FLUKA
AC-11112
AC1L1LB2
AC1Q29EZ
ACon0_000082
ACon1_000297
AI3-52939
Adasone
Ancortone
Apo-Prednisone
Apo-prednisone
BPBio1_000323
BRD-K85883481-001-04-2
BSPBio_000293
Betapar
Bicortone
Bio-0649
C07370
C21H26O5
CCRIS 2646
CHEBI:8382
CHEMBL635
CID5865
CPD001227202
Cartancyl
Colisone
Cortan
Cortancyl
Cortidelt
Cotone
DB00635
Dacorten
Dacortin
Decortancyl
Decortin
Decortisyl
Dehydrocortisone
Dekortin
Delcortin
Dellacort
Dellacort A
Delta Cortelan
Delta E
Delta E.
Delta-Cortelan
Delta-Dome
Delta-cortelan
Delta-cortisone
Delta-cortone
Delta-dome
Deltacortene
Deltacortisone
Deltacortone
Deltasone
Deltasone, Liquid Pred, Orasone, Adasone, Deltacortisone,Prednisone
Deltison
Deltisona
Deltisone
Deltra
Di-Adreson
Diadreson
EINECS 200-160-3
Econosone
Encorton
Encortone
Enkortolon
Enkorton
Fernisone
Fiasone
HMS1568O15
HMS2090J13
HSDB 3168
Hostacortin
In-Sone
Incocortyl
 
Juvason
Kortancyl
LMST02030180
LS-1325
Liquid Pred
Lisacort
Lodotra
MEGxm0_000443
MLS001061265
MLS001304073
MLS001335907
MLS001335908
MLS002154191
MLS002207083
Me-Korti
Metacortandracin
Meticorten
Meticorten (Veterinary)
Metrevet (Veterinary)
MolPort-001-740-041
NCGC00090766-01
NCGC00090766-02
NCGC00090766-03
NCI-C04897
NCI60_000008
NSC 10023
NSC10023
Nisona
Nizon
Novoprednisone
Nurison
Orasone
Origen Prednisone
P1276
P6254_SIGMA
PRD
Panafcort
Panasol
Paracort
Parmenison
Pehacort
Precort
Predeltin
Prednicen-M
Prednicorm
Prednicort
Prednicot
Prednidib
Prednilonga
Prednison
Prednisona
Prednisona [INN-Spanish]
Prednisone
Prednisone Intensol
Prednisone [INN:BAN]
Prednisonum
Prednisonum [INN-Latin]
Prednitone
Prednizon
Prednovister
Presone
Prestwick0_000077
Prestwick1_000077
Prestwick2_000077
Prestwick3_000077
Prestwick_405
Pronison
Pronisone
Rectodelt
Retrocortine
S1622_Selleck
SAM002264641
SK-Prednisone
SMR000718760
SMR001227202
SPBio_002214
Servisone
Sone
Sterapred
Supercortil
U 6020
UNII-VB0R961HZT
Ultracorten
Ultracortene
WLN: L E5 B666 CV OV AHTTT&J A1 E1 FV1Q FQ
Winpred
Wojtab
ZINC03875357
Zenadrid
Zenadrid (veterinary)
Zenadrid [veterinary]
delta cortelan
delta(sup 1)-Cortisone
delta(sup 1)-Dehydrocortisone
delta-1-Cortisone
delta-1-Dehydrocortisone
delta-Cortisone
delta-Cortone
8
Tacrolimusapproved, investigational1102104987-11-3445643, 439492
Synonyms:
(-)-FK 506
(3S,4R,5S,8R,9E,12S,14S,15R,16S,18R,19R,26aS)-5,19-dihydroxy-3-{(1E)-1-[(1R,3R,4R)-4-hydroxy-3-methoxycyclohexyl]prop-1-en-2-yl}-14,16-dimethoxy-4,10,12,18-tetramethyl-8-(prop-2-en-1-yl)-5,6,8,11,12,13,14,15,16,17,18,19,24,25,26,26a-hexadecahydro-3H-15,19-epoxypyrido[2,1-c][1,4]oxazacyclotricosine-1,7,20,21(4H,23H)-tetrone
104987-11-3
109581-93-3 (Hydrate)
15,19-epoxy-3H-pyrido[2,1-c][1,4]oxaazacyclotricosine-1,7,20,21(23H)-tetrone,
3S-[3R*[E(1S*,3S*,4S*)],4S*,5R*,8S*,9E,12R*,14R*,15S*,16R*,18S*,19S*,26aR*]]-5,6,8,11,12,13,14,15,16,17,18,19,24,25,26,26a-hexadecahydro-5, 19-dihydroxy-3-[2-(4-hydroxy-3-methoxycyclohexyl)-1-methylethenyl]-14,16-dimethoxy-4,10,12,18-tetramethyl-8-(2-propenyl)-15,19-epoxy-3H-pyrido[2,1-c] [1,4] oxaazacyclotricosine-1,7,20,21(4H,23H)-tetrone
8-DEETHYL-8-[BUT-3-ENYL]-ASCOMYCIN
AC-1182
AC1L1K7H
AC1L97GB
AC1L9IBU
AKOS005145901
Advagraf
Ambap104987-11-3
Ambap5429
Anhydrous tacrolimus
BCBcMAP01_000194
BRD-K35452788-001-02-1
BSPBio-001279
BSPBio_001279
Bio-0921
Bio2_000470
Bio2_000950
C01375
C44H69NO10
CCRIS 7124
CHEBI:100924
CHEBI:61049
CHEMBL1200738
CID11158639
CID11556866
CID439492
CID445643
CID5372
CID5472317
CID6426916
CID6436007
CID6473866
CID6536850
CID6610362
CID6912836
CID9832283
CID9853905
CID9918805
CID9940643
CID9963169
CPD-10016
CPD000466356
D08556
DB00864
DivK1c_001040
FK 506
FK-506
FK5
FK506
FR 900506
FR-900506
 
FR900506
FT-0082660
Fk-506
Fujimycin
Graceptor
HMS1362O21
HMS1792O21
HMS1990O21
HMS2051C18
HMS2093M19
HMS503O21
IDI1_001040
IDI1_002225
K506
KBio1_001040
KBio2_000619
KBio2_003187
KBio2_005755
KBio3_001097
KBio3_001098
KBioGR_000619
KBioSS_000619
L 679934
L-679934
LCP-Tacro
LMPK04000003
LS-64247
MLS000759471
MLS001424054
Modigraf
MolPort-003-666-518
NCGC00163470-01
NCGC00163470-02
NCGC00163470-03
NCGC00163470-04
NCGC00179232-01
NChemBio.2007.16-comp1
NINDS_001040
NSC717865
Prograf
Prograf (TN)
Protopic
Protopy
S5003_Selleck
SAM001246677
SMR000466356
Tacarolimus
Tacrolimus (INN)
Tacrolimus (Prograf?)
Tacrolimus (anhydrous)
Tacrolimus anhydrous
Tacrolimus hydrate
Tacrolimus, anhydrous
Tsukubaenolide
UNII-Y5L2157C4J
nchembio.2007.23-comp2
tacrolimus
tacrolimus hydrate
9Immunosuppressive Agents13086
10Calcineurin Inhibitors1622
11Anticonvulsants2695

Interventional clinical trials:

idNameStatusNCT IDPhase
1IVIG Treatment for Refractory Immune-Related Adult EpilepsyTerminatedNCT01545518Phase 2
2Immunotherapy of the Paraneoplastic SyndromesCompletedNCT00378326
3Antibodies Causing Epilepsy Syndromes: The ACES Study.RecruitingNCT02802475
4Interest Balance Auto-immune Population in Patients With Focal Epilepsy of Unknown Cause, Not Structural, Not Genetics, Newly DiagnosedRecruitingNCT02885207

Search NIH Clinical Center for Limbic Encephalitis

Genetic Tests for Limbic Encephalitis

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Anatomical Context for Limbic Encephalitis

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MalaCards organs/tissues related to Limbic Encephalitis:

36
Brain, Testes, Lung, Temporal lobe, Bone marrow, Prostate, Breast

Publications for Limbic Encephalitis

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Articles related to Limbic Encephalitis:

(show top 50)    (show all 460)
idTitleAuthorsYear
1
Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis. (28363946)
2017
2
Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. (28062719)
2017
3
Do we need to measure specific antibodies in patients with limbic encephalitis? (28062718)
2017
4
The major histocompatibility complex and antibody-mediated limbic encephalitis. (28026032)
2016
5
Editorial: Limbic Encephalitis: Autoimmune Impact on Inhibitory GABAergic Neuronal Pathways in Temporal Lobe Epilepsy. (27014186)
2016
6
Alternating faciobrachial dystonic seizures in LGI1-antibody limbic encephalitis. (26984950)
2016
7
Anti-leucine-rich glioma-inactivated 1 limbic encephalitis: A case report and literature review. (26889260)
2016
8
Limbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature. (27030381)
2016
9
Teaching NeuroImages: Limbic encephalitis associated with relapsing polychondritis. (27185902)
2016
10
Suspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy. (26940288)
2016
11
Limbic encephalitis associated with glutamic acid decarboxylase antibodies in a young adolescent. (27117742)
2016
12
Limbic encephalitis: The great unknown. (27448690)
2016
13
Limbic encephalitis associated with anti-leucine-rich glioma-inactivated-1 protein antibodies in a child. (27841212)
2016
14
Seizure control and cognitive improvement via immunotherapy in late onset epilepsy patients with paraneoplastic versus GAD65 autoantibody-associated limbic encephalitis. (27855355)
2016
15
Limbic encephalitis following immunotherapy against metastatic malignant melanoma. (27009198)
2016
16
Paraneoplastic Limbic Encephalitis in a Male with Nasopharyngeal Carcinoma. (27174340)
2016
17
A case of neurosyphilis presenting with limbic encephalitis. (27980287)
2016
18
Paraneoplastic limbic encephalitis in B cell lymphoma unclassifiable with features intermediate between diffuse large B cell lymphoma and classical Hodgkin lymphoma. (26984106)
2016
19
Evidence of a pathogenic role for CD8(+) T cells in anti-GABAB receptor limbic encephalitis. (27213174)
2016
20
Correlations of Clusters of Non-Convulsive Seizure and Magnetic Resonance Imaging in a Case With GAD65-Positive Autoimmune Limbic Encephalitis. (27429684)
2016
21
Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort. (27037228)
2016
22
Autoimmune Limbic Encephalitis and Syndrome of Inappropriate Antidiuretic Hormone Secretion Associated with Lamotrigine-induced Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome. (27181555)
2016
23
Autoimmune limbic encephalitis due to VGKC-Ab. Thanks for the memory. Leo Robin (Title of song, 1937). (26819188)
2016
24
Non-paraneoplastic limbic encephalitis and central nervous HHV-6B reactivation: Causality or coincidence? (27431532)
2016
25
Neuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis. (27566001)
2016
26
Clinical characterization of autoimmune LGI1 antibody limbic encephalitis. (26896820)
2016
27
A unique combination of autoimmune limbic encephalitis, type 1 diabetes, and Stiff person syndrome associated with GAD-65 antibody. (27011652)
2016
28
Clinical analysis of leucine-rich glioma inactivated-1 protein antibody associated with limbic encephalitis onset with seizures. (27428233)
2016
29
Type 1 diabetes and GAD65 limbic encephalitis: a case report of a 10-year-old girl. (27115322)
2016
30
Cholangiocarcinoma associated with limbic encephalitis and early cerebral abnormalities detected by 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography-positron emission tomography: a case report. (27439460)
2016
31
Limbic Encephalitis Associated with SjAPgren's Syndrome: Report of Three Cases. (27523010)
2016
32
The clinical diagnosis and treatment about 22 cases of limbic encephalitis were retrospectively analyzed. (27113304)
2016
33
Voltage-gated potassium channel antibody limbic encephalitis: a case illustrating the neuropsychiatric and PET/CT features with clinical and imaging follow-up. (27590077)
2016
34
Anti NMDA receptor encephalitis( limbic encephalitis) with corticospinal involvement in a middle aged Indian female. (27728547)
2016
35
Hyperfamiliarity for unknown faces after non-paraneoplastic limbic encephalitis: a case report. (27334285)
2016
36
Cognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis. (26881156)
2016
37
Autoimmune neurological syndromes associated limbic encephalitis and paraneoplastic cerebellar degeneration. (27592059)
2016
38
Paraneoplastic Limbic Encephalitis in a Human Epidermal Growth Factor Receptor-2-positive Gastric Cancer Patient Treated with Trastuzumab-combined Chemotherapy: A Case Report and Literature Review. (27629954)
2016
39
Small-Cell Lung Cancer with Positive Anti-NMDAR and Anti-AMPAR Antibodies Paraneoplastic Limbic Encephalitis. (28070431)
2016
40
Reversible dementia: two nursing home patients with voltage-gated potassium channel antibody-associated limbic encephalitis. (26170033)
2015
41
Clinico-pathological correlation in adenylate kinase 5 autoimmune limbic encephalitis. (26439959)
2015
42
Limbic Encephalitis: Potential Impact of Adaptive Autoimmune Inflammation on Neuronal Circuits of the Amygdala. (26284026)
2015
43
Squamous Cell Lung Carcinoma Presenting With Refractory Status Epilepticus: A Case Report of Paraneoplastic Limbic Encephalitis. (25784771)
2015
44
Differences in epileptic symptoms depending on the type of autoimmune-mediated limbic encephalitis. (26163176)
2015
45
A Rare Case of Ovarian Cancer Presenting with Paraneoplastic Cerebellar Degeneration and Limbic Encephalitis. (26415811)
2015
46
Limbic encephalitis associated with leucine-rich glioma-inactivated 1 antibodies. (26142944)
2015
47
The Effects of Carbamazepine and Lithium Combination on Serious Aggression and Anger Outbursts in a Case of Seronegative Limbic Encephalitis. (26835170)
2015
48
Treating a GAD65 Antibody-Associated Limbic Encephalitis with Basiliximab: A Case Study. (26284025)
2015
49
Multicentric Castleman's disease with voltage-gated potassium channel antibody-positive limbic encephalitis: a case report. (25648431)
2015
50
Autoimmune limbic encephalitis detected on FDG brain scan performed for the evaluation of dementia. (25608163)
2015

Variations for Limbic Encephalitis

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Expression for genes affiliated with Limbic Encephalitis

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Search GEO for disease gene expression data for Limbic Encephalitis.

Pathways for genes affiliated with Limbic Encephalitis

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GO Terms for genes affiliated with Limbic Encephalitis

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Cellular components related to Limbic Encephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1clathrin-sculpted gamma-aminobutyric acid transport vesicle membraneGO:006120210.5GAD1, GAD2
2inhibitory synapseGO:006007710.5GAD1, GAD2
3dendritic spine membraneGO:003259110.1GRIA1, PPP1R9B
4cell junctionGO:00300549.5AMPH, GAD2, GRIA1, GRIN2B, LGI1
5synapseGO:00452028.6AMPH, GAD1, GAD2, GRIA1, GRIN2B, LGI1

Biological processes related to Limbic Encephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1carboxylic acid metabolic processGO:001975210.5GAD1, GAD2
2glutamate decarboxylation to succinateGO:000654010.4GAD1, GAD2
3neurotransmitter biosynthetic processGO:004213610.3GAD1, GAD2
4ionotropic glutamate receptor signaling pathwayGO:003523510.2GRIA1, GRIN2B
5nucleobase-containing small molecule interconversionGO:00159499.8AK5, GSR
6chemical synaptic transmissionGO:00072689.4AMPH, GAD1, GAD2, GRIA1, GRIN2B

Molecular functions related to Limbic Encephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1carboxy-lyase activityGO:001683110.5GAD1, GAD2
2glutamate bindingGO:001659510.5GAD1, GAD2
3glutamate decarboxylase activityGO:000435110.5GAD1, GAD2
4extracellular-glutamate-gated ion channel activityGO:000523410.3GRIA1, GRIN2B
5ionotropic glutamate receptor activityGO:000497010.3GRIA1, GRIN2B
6lyase activityGO:00168299.7ENO1, GAD1, GAD2

Sources for Limbic Encephalitis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet