MCID: PLM006
MIFTS: 42

Pulmonary Alveolar Proteinosis malady

Categories: Respiratory diseases, Rare diseases, Blood diseases

Aliases & Classifications for Pulmonary Alveolar Proteinosis

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Aliases & Descriptions for Pulmonary Alveolar Proteinosis:

Name: Pulmonary Alveolar Proteinosis 10 47 12 36 24 65

Classifications:



External Ids:

Disease Ontology10 DOID:12120
ICD1027 J84.01
ICD9CM29 516.0
SNOMED-CT59 10501004
MeSH36 D011649
NCIt42 C85037
UMLS65 C0034050

Summaries for Pulmonary Alveolar Proteinosis

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Disease Ontology:10 A lung disease that is characterized by abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange.

MalaCards based summary: Pulmonary Alveolar Proteinosis is related to respiratory failure and myelodysplastic syndrome. An important gene associated with Pulmonary Alveolar Proteinosis is SFTPB (Surfactant Protein B), and among its related pathways are Regulation of retinoblastoma protein and Cytokines and Inflammatory Response. Affiliated tissues include lung, myeloid and bone, and related mouse phenotype respiratory system.

Wikipedia:68 Pulmonary alveolar proteinosis (abbreviated PAP), is a rare lung disease in which abnormal accumulation... more...

Related Diseases for Pulmonary Alveolar Proteinosis

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Diseases in the Pulmonary Alveolar Proteinosis family:

Acquired Pulmonary Alveolar Proteinosis Congenital Pulmonary Alveolar Proteinosis
Hereditary Pulmonary Alveolar Proteinosis Secondary Pulmonary Alveolar Proteinosis

Diseases related to Pulmonary Alveolar Proteinosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 411)
idRelated DiseaseScoreTop Affiliating Genes
1respiratory failure30.6SFTPB, SFTPC
2myelodysplastic syndrome29.8CSF1, CSF2, GATA2
3acquired pulmonary alveolar proteinosis12.9
4congenital pulmonary alveolar proteinosis12.8
5hereditary pulmonary alveolar proteinosis12.7
6severe early-onset pulmonary alveolar proteinosis due to mars deficiency12.7
7secondary pulmonary alveolar proteinosis12.7
8interstitial lung and liver disease12.0
9surfactant metabolism dysfunction, pulmonary, 111.9
10surfactant metabolism dysfunction, pulmonary, 511.9
11surfactant metabolism dysfunction, pulmonary, 411.9
12pulmonary surfactant metabolism dysfunction11.7
13surfactant metabolism dysfunction, pulmonary, 211.6
14surfactant metabolism dysfunction, pulmonary, 311.6
15abca3-related pulmonary surfactant metabolism dysfunction11.6
16csf2ra-related pulmonary surfactant metabolism dysfunction11.6
17sftpb-related pulmonary surfactant metabolism dysfunction11.6
18sftpc-related pulmonary surfactant metabolism dysfunction11.6
19immunodeficiency 2110.8
20lysinuric protein intolerance10.6
21leukemia10.6
22breast cancer10.5
23prostatitis10.5
24hepatitis10.5
25endotheliitis10.5
26adenocarcinoma10.4
27schizophrenia10.4
28myocardial infarction10.4
29prostate cancer10.4
30obesity10.4
31lymphoma10.4
32cervicitis10.4
33cerebritis10.4
34retinitis10.4
35thyroiditis10.4
36neuronitis10.4
37congenital lactic acidosis, saguenay-lac-saint-jean type10.3SFTPB, SFTPC
38transcobalamin deficiency10.3SFTPB, SFTPC
39niemann-pick disease10.3SFTPB, SFTPC
40colorectal cancer10.3
41hepatocellular carcinoma10.3
42acute myocardial infarction10.3
43liver disease10.3
44colitis10.3
45giardiasis10.3
46melanoma10.3
47pancreatitis10.3
48esophageal atresia10.3SFTPB, SFTPC
49acute zonal occult outer retinopathy10.3SFTPB, SFTPC
50neuroblastoma10.2

Graphical network of the top 20 diseases related to Pulmonary Alveolar Proteinosis:



Diseases related to pulmonary alveolar proteinosis

Symptoms for Pulmonary Alveolar Proteinosis

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Drugs & Therapeutics for Pulmonary Alveolar Proteinosis

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Drugs for Pulmonary Alveolar Proteinosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 15)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1molgramostimPhase 2, Phase 3, Phase 112
2Pharmaceutical SolutionsPhase 2, Phase 3, Phase 17004
3Pulmonary SurfactantsPhase 2, Phase 3101
4AutoantibodiesPhase 2, Phase 3, Phase 1117
5Antibiotics, AntitubercularPhase 2, Phase 35971
6Anti-Bacterial AgentsPhase 2, Phase 39140
7
rituximabapprovedPhase 2, Phase 11582174722-31-710201696
Synonyms:
AntiCD20
IDEC-102
IDEC-C2B8
 
Ig gamma-1 chain C region
MabThera
Mabthera
Rituxan
rituximab
8Immunologic FactorsPhase 2, Phase 118483
9Antirheumatic AgentsPhase 2, Phase 18496
10AntibodiesPhase 2, Phase 14477
11ImmunoglobulinsPhase 2, Phase 14477
12Antibodies, MonoclonalPhase 12413
13
Nicotineapproved97354-11-5942, 89594
Synonyms:
(−
(+)-Nicotine
(+-)-3-(1-Methyl-2-pyrrolidinyl)pyridine
(+-)-Nicotine
(-)-3-(1-Methyl-2-pyrrolidyl)pyridine
(-)-3-(N-Methylpyrrolidino)pyridine
(-)-Nicotine
(-)-Nicotine solution
(2S) 3-(1-Methyl-pyrrolidin-2-yl)-pyridine
(R)-3-(1-Methyl-2-pyrrolidinyl)pyridine
(R,S)-Nicotine
(R,S)-nicotine
(RS)-nicotine
(S)-(-)-NICOTINE, 3-[(2S)-1-METHYL-2-PYRROLIDINYL] PYRIDINE
(S)-(-)-Nicotine
(S)-3-(1-methylpyrrolidin-2-yl)pyridine
(S)-3-(N-methylpyrrolidin-2-yl)pyridine
(S)-Nicotine
(┬▒)-nicotine
)-1-Methyl-2-(3-pyridyl)pyrrolidine
)-Nicotine
)-Nicotine solution
1-Methyl-2-(3-pyridal)-Pyrrolidine
1-Methyl-2-(3-pyridal)-pyrrolidene
1-Methyl-2-(3-pyridiyl)pyrrolidine
1-Methyl-2-(3-pyridyl)pyrrolidine
1uw6
2'-beta-H-Nicotine
3-(1-Methyl-2-pyrollidinyl)pyridine
3-(1-Methyl-2-pyrrolidinyl)-Pyridine
3-(1-Methyl-2-pyrrolidinyl)pyridine
3-(1-Methylpyrrolidin-2-yl)pyridine
3-(2-(N-methylpyrrolidinyl))pyridine
3-(N-Methylpyrollidino)pyridine
3-(N-Methylpyrrolidino)pyridine
3-N-Methylpyrrolidine
3-[(2S)-1-methylpyrrolidin-2-yl]pyridine
36733_FLUKA
36733_RIEDEL
434F7990-3240-4A43-ACEC-E6CC1E495FA0
46343_FLUKA
46343_RIEDEL
54-11-5
AC1L3I79
AC1Q3ZOC
AI3-03424
American Fare Nicotine Transdermal System
BB_NC-0777
BIDD:GT0599
BRD-K05395900-322-02-1
Black Leaf
Black Leaf 40
C00745
CCRIS 1637
CHEBI:17688
CHEMBL3
CID89594
CPD000059074
Campbell's Nico-Soap
Careone Nicotine Transdermal System Step 1
Careone Nicotine Transdermal System Step 2
Careone Nicotine Transdermal System Step 3
Caswell No. 597
Commit
D-Nicotine
D03365
DL-tetrahydronicotyrine
Destruxol
Destruxol Orchid Spray
EINECS 200-193-3
ENT 3,424
EPA Pesticide Chemical Code 056702
Emo-Nik
Equaline Nicotine Transdermal System STEP 1
Exchange Select Nicotine Transdermal System STEP 3
Flux Maag
Fumeto bac
Fumetobac
HSDB 1107
Habitrol
Habitrol (TN)
Habitrol Patch Nicotine Transdermal System, Step 1
Habitrol Patch Nicotine Transdermal System, Step 2
Habitrol Patch Nicotine Transdermal System, Step 3
Healthmart Nicotine Transdermal System STEP 1
Healthmart Nicotine Transdermal System STEP 2
Healthmart Nicotine Transdermal System STEP 3
Kroger Nicotine Transdermal System STEP 1
Kroger Nicotine Transdermal System STEP 2
Kroger Nicotine Transdermal System STEP 3
L(-)-nicotine
L-3-(1-Methyl-2-pyrrolidyl)pyridine
L-Nicotine
LS-202
Leader Nicotine
Leader Nicotine Transdermal System STEP 1
Leader Nicotine Transdermal System STEP 2
Leader Nicotine Transdermal System STEP 3
MLS001055457
 
MLS001335905
Mach-Nic
Meijer Nicotine Transdermal System STEP 2
Meijer Nicotine Transdermal System Step 1
Meijer Nicotine Transdermal System Step 3
Methyl-2-pyrrolidinyl)pyridine
Micotine
MolPort-000-744-731
N3876_SIGMA
N5511_FLUKA
N5511_SIGMA
NCGC00090693-01
NCGC00090693-02
NCGC00090693-03
NCGC00090693-04
NCGC00090693-05
NCGC00090693-06
NCGC00090693-07
NCT
NICOTINE AND SALTS
NSC 5065
NSC97238
Niagara P.A. Dust
Nic-Sal
Nico-Dust
Nico-Fume
Nicocide
Nicoderm
Nicoderm CQ
Nicoderm Cq
Nicoderm Patch
Nicorette
Nicorette Plus
Nicotin
Nicotina
Nicotina [Italian]
Nicotine
Nicotine (USP)
Nicotine (compounds related to)
Nicotine Alkaloid
Nicotine Patch
Nicotine Polacrilex
Nicotine TRANSDERMAL SYSTEM
Nicotine Transdermal System PATCH KIT
Nicotine Transdermal System STEP 1
Nicotine Transdermal System STEP 2
Nicotine Transdermal System STEP 3
Nicotine Transdermal System Step 1
Nicotine Transdermal System Step 2
Nicotine Transdermal System Step 3
Nicotine [BSI:ISO]
Nicotine [UN1654]
Nicotine [UN1654] [Poison]
Nicotine [USAN]
Nicotine transdermal system
Nicotrol
Nicotrol (professional Sample)
Nicotrol Inhaler
Nicotrol Ns
Nictoine patch
Nikotin
Nikotin [German]
Nikotyna
Nikotyna [Polish]
Ortho N-4 Dust
Ortho N-5 Dust
PDSP1_000113
PDSP1_000465
PDSP2_000463
PDSP2_000555
Prostep
R)-(+)-Nicotine
RCRA waste no. P075
RCRA waste number P075
Rite Aid Nicotine Transdermal System Step 1
Rite Aid Nicotine Transdermal System Step 2
Rite Aid Nicotine Transdermal System Step 3
SAM002564224
SDCCGMLS-0066911.P001
SMR000059074
Safeway Nicotine Transdermal System STEP 1
Safeway Nicotine Transdermal System STEP 2
Safeway Nicotine Transdermal System STEP 3
Smart Sense Nicotine
Sunmark Nicotine
Sunmark Nicotine Transdermal System STEP 1
Sunmark Nicotine Transdermal System STEP 2
Sunmark Nicotine Transdermal System STEP 3
Tendust
Transdermal Nicotine
UN1654
a -N-Methylpyrrolidine
a-N-Methylpyrrolidine
alpha-N-Methylpyrrolidine
beta-Pyridyl-alpha-N-methyl pyrrolidine
beta-Pyridyl-alpha-N-methylpyrrolidine
bmse000105
delta-Nicotine
nicotine
nicotine replacement patch
14Respiratory System Agents3931
15Protein C86

Interventional clinical trials:

(show all 13)
idNameStatusNCT IDPhase
1Efficacy and Safety of Inhaled Molgramostim (rhGM-CSF) in Autoimmune Pulmonary Alveolar Proteinosis (aPAP)RecruitingNCT02702180Phase 2, Phase 3
2Whole Lung Lavage (WLL)/Inhaled Granulocyte-macrophage Colony-stimulating Factor (GM-CSF) in Autoimmune Pulmonary Alveolar Proteinosis (PAP)Enrolling by invitationNCT00901511Phase 2, Phase 3
3Inhaled Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) in Hereditary Pulmonary Alveolar Proteinosis (PAP)CompletedNCT01511068Phase 2
4Prospective Trial of Rituximab for Primary Pulmonary Alveolar ProteinosisRecruitingNCT00552461Phase 2
5Inhalation of Granulocyte-macrophage Colony-stimulating Factor (GM-CSF) for Autoimmune Pulmonary Alveolar Proteinosis (PAP)RecruitingNCT02243228Phase 2
6Study of Subcutaneous Injection of Low-dose rhGM-CSF +/- WLL in PAP.RecruitingNCT01983657Phase 2
7GM-CSF in Patients With Pulmonary Alveolar ProteinosisTerminatedNCT00030056Phase 2
8Inhaled Molgramostim (rhGM-CSF) in Healthy Adult SubjectsCompletedNCT02468908Phase 1
9Rituximab for Anti-cytokine Autoantibody-Associated DiseasesRecruitingNCT01842386Phase 1
10A National Registry For Pulmonary Alveolar ProteinosisRecruitingNCT02461615
11Evaluation and Treatment Planning of Patients With PAP Using Thrive Ultra Short Echo Time MRI and CTRecruitingNCT02081092
12Natural History Study of GATA2 Deficiency and Related DisordersRecruitingNCT01905826
13Epidemiology of Surfactant Protein-B DeficiencyRecruitingNCT00014859

Search NIH Clinical Center for Pulmonary Alveolar Proteinosis


Cochrane evidence based reviews: pulmonary alveolar proteinosis

Genetic Tests for Pulmonary Alveolar Proteinosis

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Anatomical Context for Pulmonary Alveolar Proteinosis

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MalaCards organs/tissues related to Pulmonary Alveolar Proteinosis:

33
Lung, Myeloid, Bone, T cells, Liver, Thyroid, Endothelial

Animal Models for Pulmonary Alveolar Proteinosis or affiliated genes

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MGI Mouse Phenotypes related to Pulmonary Alveolar Proteinosis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.4CSF1, CSF2, SFTPB, SFTPC, SFTPD

Publications for Pulmonary Alveolar Proteinosis

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Articles related to Pulmonary Alveolar Proteinosis:

(show top 50)    (show all 560)
idTitleAuthorsYear
1
Postpartum thyroid dysfunction in women with autoimmune thyroiditis. (26669901)
2016
2
Genetic analysis of FMR1 repeat expansion in essential tremor. (25796179)
2015
3
Legionella prevalence and risk of legionellosis in Hungarian hospitals. (26689882)
2015
4
Reduced sTWEAK and increased sCD163 levels in HIV-infected patients: modulation by antiretroviral treatment, HIV replication and HCV co-infection. (24594990)
2014
5
CCR5 Plays Important Roles in Hepatitis B Infection. (24405101)
2014
6
Substance abuse journal: new beginnings. (24159902)
2013
7
Hypertrophic pachymeningitis in an individual with microscopic polyangiitis. (23772738)
2013
8
Orbital aspects following monobloc advancement in syndromic craniosynostosis. (23352809)
2013
9
Lipoic acid in animal models and clinical use in diabetic retinopathy. (23790257)
2013
10
A failure in endothelin-1 production from vitiligo keratinocytes in response to ultraviolet B irradiation. (23815989)
2013
11
Sensitivity of TRPM7 channels to Mg2+ characterized in cell-free patches of Jurkat T lymphocytes. (22460708)
2012
12
Overexpression of Golgi phosphoprotein-3 (GOLPH3) in glioblastoma multiforme is associated with worse prognosis. (22972189)
2012
13
Exfoliative esophagitis and esophageal ulcer induced by dabigatran. (22396259)
2012
14
Sesquiterpene lactone-related allergic contact dermatitis after exposure to tulip poplar wood and bark. (22653126)
2012
15
Serotonin potentiates transforming growth factor-beta3 induced biomechanical remodeling in avian embryonic atrioventricular valves. (22880017)
2012
16
GPR56 Regulates VEGF production and angiogenesis during melanoma progression. (21724588)
2011
17
Study of serum ghrelin changes and its correlation with malnutrition in liver cirrhosis in Egypt. (21872552)
2011
18
Paradoxical hepatic tumor: Undifferentiated embryonal sarcoma of the liver. (20352000)
2010
19
Relaxin stimulates osteoclast differentiation and activation. (19833242)
2010
20
Ribonuclear inclusions and MBNL1 nuclear sequestration do not affect myoblast differentiation but alter gene splicing in myotonic dystrophy type 2. (19345584)
2009
21
Isoform-specific contributions of alpha-actinin to glioma cell mechanobiology. (20037648)
2009
22
Human pre-elafin inhibits a Pseudomonas aeruginosa-secreted peptidase and prevents its proliferation in complex media. (18025118)
2008
23
The postsynaptic density 95/disc-large/zona occludens protein syntenin directly interacts with frizzled 7 and supports noncanonical Wnt signaling. (18256285)
2008
24
C/EBPalpha and C/EBPvarepsilon induce the monocytic differentiation of myelomonocytic cells with the MLL-chimeric fusion gene. (18776924)
2008
25
Detection of CD4(+) T-cell antibodies in a patient with idiopathic CD4 T lymphocytopenia and cryptococcal meningitis. (17854318)
2007
26
Pemphigus vulgaris and pemphigus foliaceus: similar prognosis? (17822493)
2007
27
Atypical herpes simplex infection masquerading as recalcitrant pemphigus vulgaris. (17956485)
2007
28
Protein kinase C delta (deltaPKC)-annexin V interaction: a required step in deltaPKC translocation and function. (16785226)
2006
29
Synchronous collecting duct carcinoma and papillary renal cell carcinoma: a case report and review of the literature. (15816631)
2005
30
Serum levels of leptin and proinflammatory cytokines in advanced-stage non-small cell lung cancer. (16260852)
2005
31
Human and simian immunodeficiency virus capsid proteins are major viral determinants of early, postentry replication blocks in simian cells. (12477877)
2003
32
Evaluation of vitrectomy specimens and chorioretinal biopsies in the diagnosis of primary intraocular lymphoma in patients with Masquerade syndrome. (14605902)
2003
33
DNA mismatch repair (MMR) mediates 6-thioguanine genotoxicity by introducing single-strand breaks to signal a G2-M arrest in MMR-proficient RKO cells. (12796402)
2003
34
Inflammatory neurodegeneration mediated by nitric oxide from activated glia-inhibiting neuronal respiration, causing glutamate release and excitotoxicity. (11517237)
2001
35
Giardiasis-associated arthralgia in children. (11471528)
2001
36
Inflammation in the developing human intestine: A possible pathophysiologic contribution to necrotizing enterocolitis. (10823949)
2000
37
U373-MG response to interleukin-1beta-induced oxidative stress. (11032359)
2000
38
Finite lifespans of T cell clones derived from CD34+ human haematopoietic stem cells in vitro. (10197729)
1999
39
Molecular cloning and sequence analysis of the cDNA for the mouse counterpart to adult hamster liver purified growth inhibitory factor. (9060991)
1997
40
Unchanged iron and copper and increased zinc in the blood of obese children after two hypocaloric diets. (9282256)
1997
41
Cutaneous fibrous histiocytoma with osteoclast-like giant cells. (8863988)
1996
42
Lifetime DSM-IV diagnosis of alcohol, cannabis, cocaine and opiate dependence: six-month reliability in a multi-site clinical sample. (7987188)
1994
43
Rab6 is associated with a compartment that transports rhodopsin from the trans-Golgi to the site of rod outer segment disk formation in frog retinal photoreceptors. (8308063)
1993
44
Zinc finger-like structure in U1-specific protein C is essential for specific binding to U1 snRNP. (1826349)
1991
45
Treatment of late infantile metachromatic leukodystrophy by bone marrow transplantation. (1967188)
1990
46
Cervical involvement of diffuse idiopathic skeletal hyperostosis with dysphagia and rhinolalia. (3266838)
1988
47
Cloning and expression of two human genes encoding calcium-binding proteins that are regulated during myeloid differentiation. (3405210)
1988
48
Mechanism of nerve destruction in tuberculoid-borderline leprosy. An electron-microscopic study. (4582968)
1973
49
50

Variations for Pulmonary Alveolar Proteinosis

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Expression for genes affiliated with Pulmonary Alveolar Proteinosis

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Search GEO for disease gene expression data for Pulmonary Alveolar Proteinosis.

Pathways for genes affiliated with Pulmonary Alveolar Proteinosis

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Pathways related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

(show all 11)
idSuper pathwaysScoreTop Affiliating Genes
19.7CSF2, SFTPD
29.5CSF1, CSF2
3
Show member pathways
9.4SFTPB, SFTPC, SFTPD
49.0CCL2, CSF1
59.0CCL2, CSF1
69.0CSF1, CSF2, CSF2RA
7
Show member pathways
8.9CSF2RA, SFTPB, SFTPC, SFTPD
8
Show member pathways
8.9CSF2RA, SFTPB, SFTPC, SFTPD
9
Show member pathways
8.9CSF2RA, SFTPB, SFTPC, SFTPD
108.7CCL2, CSF1, CSF2
118.7CCL2, CSF1, CSF2

GO Terms for genes affiliated with Pulmonary Alveolar Proteinosis

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Cellular components related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1multivesicular body lumenGO:009748610.2SFTPB, SFTPC
2clathrin-coated endocytic vesicleGO:004533410.0SFTPB, SFTPD
3extracellular regionGO:00055769.4SFTPB, SFTPC, SFTPD

Biological processes related to Pulmonary Alveolar Proteinosis according to GeneCards Suite gene sharing:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of macrophage derived foam cell differentiationGO:001074410.1CSF1, CSF2
2peptidyl-tyrosine phosphorylationGO:001810810.0CSF2, CSF2RA
3homeostasis of number of cells within a tissueGO:004887310.0CSF1, GATA2
4organ morphogenesisGO:00098879.7CCL2, SFTPB
5positive regulation of macrophage chemotaxisGO:00107599.6CCL2, CSF1
6positive regulation of GTPase activityGO:00435479.0CCL2, CSF2, CSF2RA
7innate immune responseGO:00450878.5CSF1, CSF2, CSF2RA, SFTPD

Sources for Pulmonary Alveolar Proteinosis

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet