MCID: BRN022
MIFTS: 64

Bronchiectasis malady

Summaries for Bronchiectasis

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8Disease Ontology, 63Wikipedia, 46OMIM, 32MalaCards
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Disease Ontology:8 A bronchial disease that is a chronic inflammatory condition of one or more bronchi or bronchioles marked by dilatation and loss of elasticity of the walls resulting from damage to the airway wall leading to the formation of small sacs on the bronchial wall and impairment of cilia mobility in the lung. inflammation of the bronchial wall increases mucus secretion which serves as a breeding ground for bacteria. bronchiectasis is caused by repeated respiratory infections, immune deficiency disorders, hereditary disorders (cystic fibrosis or primary ciliary dyskinesia), mechanical factors (inhaled object or a lung tumor) or inhaling toxic substances.

MalaCards: Bronchiectasis, also known as polynesian bronchiectasis, is related to cystic fibrosis and asthma. An important gene associated with Bronchiectasis is CFTR (cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)), and among its related pathways are Class C/3 (Metabotropic glutamate/pheromone receptors) and Aldosterone-regulated sodium reabsorption. The drug acetylcysteine and the compounds h2o2 and hocl have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and colon, and related mouse phenotypes are homeostasis/metabolism and immune system.

Wikipedia:63 Bronchiectasis is a disease state defined by localized, irreversible dilation of part of the bronchial... more...

Description from OMIM:46 211400

Aliases & Classifications for Bronchiectasis

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8Disease Ontology, 22GTR, 10DISEASES, 44Novoseek, 60UMLS, 56SNOMED-CT, 46OMIM, 34MeSH, 27ICD9CM, 39NCIt
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Aliases & Descriptions:

bronchiectasis 8 22 10 44 60
polynesian bronchiectasis 8


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Disease Ontology8 DOID:9563
OMIM46 211400
MeSH34 D001987
ICD9CM27 494
NCIt39 C84475

Related Diseases for Bronchiectasis

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17GeneCards, 18GeneDecks
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Diseases related to Bronchiectasis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 238)
idRelated DiseaseScoreTop Affiliating Genes
1cystic fibrosis31.3ELANE, SERPINA3, SERPINA1, SLPI, CFTR, IL8
2asthma30.6IGHE, RNASE3
3pulmonary fibrosis30.5MPO, IL8, ELANE
4aspergillosis30.4TLR2, MBL2, SLPI, ELANE
5pneumonia30.4BPI, TLR2, MBL2, CTSG, MPO, IL8
6bronchitis30.4SERPINA1, RNASE3
7allergic bronchopulmonary aspergillosis30.4CFTR, IGHE, MBL2
8tuberculosis30.3SERPINA1, CTSG, MBL2, TLR2, BPI
9alpha 1-antitrypsin deficiency30.2SERPINA1, SERPINA3, ELANE
10influenza30.2MBL2, TAP2, CFTR
11thyroiditis30.1MBL2
12hypertension30.1SCNN1B, SCNN1A, SCNN1G
13arthritis30.1CTSG, IL8, SERPINA1
14vasculitis30.1BPI, MBL2, CTSG, MPO, SERPINA1, ELANE
15rheumatoid arthritis30.1BPI, TLR2, MBL2, CTSG, MPO, RNASE3
16respiratory failure29.9SERPINA1, CFTR
17bronchiectasis with or without elevated sweat chloride 129.9SCNN1B
18glomerulonephritis29.9ELANE, MPO, CTSG, MBL2
19cholangitis29.7CFTR
20myocardial infarction29.7SERPINA3
21sarcoidosis29.7TAP2, SERPINA3
22dermatitis29.7IGHE
23bronchiolitis obliterans29.7MMP8, IL8
24bronchiolitis29.7IGHE, RNASE3, IL8
25rhinitis29.7IL8, RNASE3, IGHE
26pulmonary emphysema29.7CTSG, MMP8, SLPI, SERPINA1, ELANE
27ischemia29.7MPO
28breast cancer29.7TAP2, MMP8, CFTR, SERPINA1
29ulcerative colitis29.7BPI, TLR2, CTSG, MPO, RNASE3, IL8
30intrinsic asthma29.7IGHE, RNASE3
31primary ciliary dyskinesia10.3
32dextrocardia10.2
33yellow nail syndrome10.2
34common variable immunodeficiency10.2
35bronchiectasis with or without elevated sweat chloride 1, modifier of10.2
36bronchiectasis with or without elevated sweat chloride 210.1
37situs inversus10.1
38agammaglobulinemia10.1
39amyloidosis10.1
40aspiration pneumonia10.1
41esophagitis10.1
42obstructive lung disease10.1
43ciliary dyskinesia-bronchiectasis10.1
44bronchiectasis with or without elevated sweat chloride 310.1
45pulmonary disease, chronic obstructive10.0SERPINA1
46coronary heart disease10.0MMP8
47diabetes mellitus10.0SERPINA1
48common variable immune deficiency10.0MBL2
49cholera10.0CFTR
50acute myocardial infarction10.0SERPINA1, MMP8

Graphical network of the top 20 diseases related to Bronchiectasis:



Diseases related to bronchiectasis

Clinical Features for Bronchiectasis

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46OMIM
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Clinical features from OMIM:

211400

Drugs & Therapeutics for Bronchiectasis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Bronchiectasis

Drug clinical trials:

Search ClinicalTrials for Bronchiectasis

Search NIH Clinical Center for Bronchiectasis

Search CenterWatch for Bronchiectasis

Inferred drug relations via UMLS60/NDF-RT40:

Genetic Tests for Bronchiectasis

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22GTR
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Genetic tests related to Bronchiectasis:

id Genetic test Affiliating Genes
1 Bronchiectasis22

Anatomical Context for Bronchiectasis

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32MalaCards
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MalaCards organs/tissues related to Bronchiectasis:

32
Lung, Testes, Colon, Thyroid, Kidney, Bone, Bone marrow, Brain, Lymph node, Monocytes, T cells, B cells, Smooth muscle, Breast

Animal Models for Bronchiectasis or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Bronchiectasis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537610.2MBL2, SCNN1B, SCNN1A, SCNN1G, MPO, IGHE
2MP:000538710.0ELANE, TLR2, MBL2, CTSG, MPO, IGHE
3MP:00053979.9MPO, CTSG, MBL2, TLR2, IGHE, IL8

Publications for Bronchiectasis

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50PubMed
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Articles related to Bronchiectasis:

(show top 50)    (show all 609)
idTitleAuthorsYear
1
Bronchiectasis: an update on current pharmacotherapy and future perspectives. (24410485)
2014
2
Inhaled, dual release liposomal ciprofloxacin in non-cystic fibrosis bronchiectasis (ORBIT-2): a randomised, double-blind, placebo-controlled trial. (23681906)
2013
3
Upper and lower airways associations in patients with chronic rhinosinusitis and bronchiectasis. (23881553)
2013
4
Bronchiectasis and severe respiratory insufficiency associated with a new surfactant protein C mutation. (23025826)
2013
5
Phase II study of a neutrophil elastase inhibitor (AZD9668) in patients with bronchiectasis. (23433769)
2013
6
Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis. (23314900)
2013
7
Positron emission tomography in the prediction of inflammation in children with human immunodeficiency virus related bronchiectasis. (22413108)
2012
8
Pulmonary bacterial communities in surgically resected noncystic fibrosis bronchiectasis lungs are similar to those in cystic fibrosis. (22448327)
2012
9
Non-cystic fibrosis bronchiectasis. (21040075)
2012
10
Pulmonary resection and lung transplantation for bronchiectasis. (22640854)
2012
11
Low serum mannose-binding lectin level is not associated with disease severity in non-cystic fibrosis bronchiectasis. (22382779)
2012
12
Systemic comorbidities in bronchiectasis. (23129800)
2012
13
The initial evaluation of adults with bronchiectasis. (22640842)
2012
14
Inhaled gentamicin in non-cystic fibrosis bronchiectasis: effects of long-term therapy. (21470074)
2011
15
Immunological testing in non-cystic fibrosis bronchiectasis. (22184579)
2011
16
Factors associated with bronchiectasis in patients with COPD. (21546440)
2011
17
Pneumatosis intestinalis presenting with a pneumoperitoneum in a patient with chronic bronchiectasis: a delayed diagnosis of superior mesenteric artery ischaemia. (22767661)
2010
18
Comparative study for the virulence of Mycobacterium avium isolates from patients with nodular-bronchiectasis- and cavitary-type diseases. (20440531)
2010
19
Bronchiectasis. (20630967)
2010
20
Resolution of extensive severe bronchiectasis in an infant. (20575086)
2010
21
Bronchiectasis and immune deficiency in an adult patient with deletion 2q37 due to an unbalanced translocation t(2;10). (19272308)
2009
22
Bactericidal activity of neutrophils with reduced oxidative burst from adults with bronchiectasis. (19239435)
2009
23
Validation of the Leicester Cough Questionnaire in non-cystic fibrosis bronchiectasis. (19196812)
2009
24
Inhaled steroids for bronchiectasis. (19160186)
2009
25
Effects of combination of fluticasone propionate and salmeterol xinafoate on lung function improvement in patients with bronchiectasis. (22046624)
2009
26
Association between mucoid Pseudomonas infection and bronchiectasis in children with cystic fibrosis. (19703887)
2009
27
Congenital lymphoedema, bronchiectasis and seizure: case report. (18663889)
2008
28
Upregulation of ICAM-1 expression in bronchial epithelial cells by airway secretions in bronchiectasis. (17931847)
2008
29
Bronchiectasis-clinical cases. (18638625)
2008
30
A patient with bronchiectasis presented with progressive dyspnoea and an unresolved right lower lung infiltrate. (18820116)
2008
31
Aerosolized antibiotics for non-cystic fibrosis bronchiectasis. (18518833)
2008
32
Etiology and outcome of bronchiectasis in children: a study of 41 patients. (19213492)
2008
33
Patients with cartilage-hair hypoplasia have an increased risk for bronchiectasis. (18280853)
2008
34
Pseudomonas aeruginosa choroidal abscess in a patient with bronchiectasis. (17687520)
2008
35
Increased airway smooth muscle mass in children with asthma, cystic fibrosis, and non-cystic fibrosis bronchiectasis. (18218992)
2008
36
Bronchiectasis in adults. Foreword. (18638618)
2008
37
Exacerbations in cystic fibrosis: 4--Non-cystic fibrosis bronchiectasis. (18308962)
2008
38
Association between inflammatory markers in induced sputum and clinical characteristics in children with non-cystic fibrosis bronchiectasis. (17351928)
2007
39
Dissociation of lung function, dyspnea ratings and pulmonary extension in bronchiectasis. (17698334)
2007
40
Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency. (17872489)
2007
41
The impact of bronchiectasis in clinical presentation of asthma. (17534081)
2007
42
Association of presence of Aspergillus antibodies with hemoptysis in patients with old tuberculosis or bronchiectasis but no radiologically visible mycetoma. (14766834)
2004
43
Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis. (11877474)
2002
44
Specific antibody response against the 23-valent pneumococcal vaccine in patients with alpha(1)-antitrypsin deficiency with and without bronchiectasis. (10531158)
1999
45
Vasculitis and bronchiectasis in a patient with antibodies to bactericidal/permeability-increasing protein and alpha1-antitrypsin deficiency. (9404779)
1997
46
Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency. (8711667)
1996
47
Bronchiectasis and Felty's syndrome. (8736662)
1996
48
Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency in a young man. (7785020)
1995
49
Variable dysgammaglobulinemia and bronchiectasis. (6766917)
1980
50
Bronchiolar proliferation and metaplasia associated with bronchiectasis, pulmonary infarcts, and anthracosis. (13652097)
1959

Genetic Variations for Bronchiectasis

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Expression for genes affiliated with Bronchiectasis

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Bronchiectasis

Search GEO for disease gene expression data for Bronchiectasis.

Pathways for genes affiliated with Bronchiectasis

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53Reactome, 29KEGG, 12EMD Millipore, 49PharmGKB, 51QIAGEN
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Pathways related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 12)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.4SCNN1B, SCNN1A, SCNN1G
210.4SCNN1G, SCNN1A, SCNN1B
310.4SCNN1B, SCNN1A, SCNN1G
410.4SCNN1B, SCNN1A, SCNN1G
510.4ELANE, MMP8, CTSG
6
Hide members
10.4MBL2, FCN2
7
Hide members
10.4CFTR, SCNN1G, SCNN1A, SCNN1B
8
NO-dependent CFTR activation (normal and CF)
10.4CFTR, SCNN1G, SCNN1A, SCNN1B
9
CFTR-dependent regulation of ion channels in Airway Epithelium (norm and CF)
10.4CFTR, SCNN1G, SCNN1A, SCNN1B
10
Immune response Bacterial infections in normal airways
10.4CFTR, IL8, TLR2
1110.4IL8, SCNN1A, TLR2
1210.3TAP2, MPO, MBL2, TLR2

Compounds for genes affiliated with Bronchiectasis

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44Novoseek, 24HMDB, 28IUPHAR, 11DrugBank, 49PharmGKB, 59Tocris Bioscience
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Compounds related to Bronchiectasis according to GeneCards/GeneDecks:

(show top 50)    (show all 129)
idCompoundScoreTop Affiliating Genes
1h2o24410.8CFTR, CTSG, MMP8, MPO
2hocl4410.8SERPINA1, ELANE, MMP8, MPO, CTSG
3endotoxin4410.8SLPI, RNASE3, CTSG, BPI
4superoxide44 2411.7MBL2, MMP8, ELANE, SERPINA3, SLPI
5zymosan4410.7RNASE3, ELANE, MBL2, TLR2, MPO
6serine4410.7MMP8, SERPINA3, CTSG, MPO, IL8, TLR2
7amiloride44 28 1112.7CFTR, MPO, SCNN1B, SCNN1G, SCNN1A
8fmlp4410.7BPI, CTSG, RNASE3, SERPINA3, MPO, ELANE
9lipoteichoic acid4410.7MBL2, TLR2, IL8, BPI, SLPI
10methacholine44 1111.7MPO, CTSG, RNASE3, IL8, IGHE, SLPI
11fluticasone propionate44 49 59 1113.7IL8, IGHE, RNASE3, MPO, SLPI, ELANE
12n-formylmethionyl-leucylphenylalanine4410.7CTSG, MPO, RNASE3, IL8, ELANE
13triamterene44 11 2412.7SCNN1B, SCNN1A, SCNN1G
14urea44 11 2412.7SLPI, MMP8, SERPINA3, BPI, CTSG, RNASE3
15leukotriene b444 59 2412.7MPO, CTSG, ELANE, TLR2, RNASE3, IL8
16cyclosporin a44 28 5912.7ELANE, MMP8, SLPI, CFTR, IGHE, RNASE3
17anti-fungal4410.7SLPI, RNASE3, MBL2, TLR2, BPI
18diisopropylfluorophosphate4410.7CTSG, SERPINA1, SERPINA3, ELANE
19dexamethasone44 49 28 1113.7IGHE, TLR2, RNASE3, SLPI, CFTR, SCNN1A
20polymyxin b4410.7IL8, BPI, TLR2, MBL2
21anaphylatoxin4410.7MPO, RNASE3, ELANE, IL8
22sodium44 2411.7CTSG, SLPI, CFTR, MBL2, SCNN1B, SCNN1A
23aprotinin44 1111.6ELANE, CTSG, IGHE, IL8, SLPI
24heparin44 28 11 2413.6ELANE, SERPINA1, SLPI, SERPINA3, IL8, CTSG
25polysaccharide4410.6BPI, TLR2, MBL2, CTSG, IGHE, FCN2
26chlorine44 2411.6CFTR, ELANE, RNASE3, IGHE
27budesonide44 49 1112.6MPO, IL8, IGHE, RNASE3
28lactate4410.6ELANE, CTSG, BPI, IL8, MPO, SLPI
29fibrinogen4410.6MMP8, SLPI, SERPINA1, SERPINA3, ELANE, CTSG
30creatinine4410.6CTSG, MPO, MBL2, IGHE, RNASE3, SLPI
31actinomycin d4410.6SCNN1A, CFTR, IL8, IGHE, TLR2, BPI
32histamine44 28 2412.6SLPI, IL8, RNASE3, MPO, CTSG, TLR2
33acetylcholine44 49 28 11 2414.6ELANE, MPO, CFTR, SERPINA3
34cysteine4410.6SERPINA1, CFTR, ELANE, SCNN1G, TLR2, CTSG
35nitric oxide44 11 2412.6TLR2, BPI, SERPINA1, SLPI, MMP8, IL8
36retinoic acid44 2411.6ELANE, SERPINA3, SLPI, MMP8, RNASE3, IGHE
37alanine4410.6SLPI, BPI, IGHE, MMP8, CFTR, CTSG
38vegf4410.5RNASE3, IL8, MMP8, SLPI, IGHE, MPO
39cytochalasin b4410.5CTSG, MPO, RNASE3, ELANE
40carbohydrates4410.5CTSG, MBL2, IGHE, RNASE3, CFTR
41vitamin d4410.5TLR2, ELANE, SLPI, CFTR, IGHE, MBL2
42cilomilast4410.5ELANE, IL8, MPO
43ethanol44 49 11 2413.5TLR2, MBL2, SLPI, BPI, RNASE3, SERPINA1
44oxygen44 2411.5MPO, IL8, SLPI, SERPINA1, ELANE, MMP8
45peptidoglycan44 2811.5MBL2, TLR2, IL8, CTSG
46chloramine t4410.4MPO, ELANE, SLPI
47valine4410.4CFTR, SERPINA3, ELANE
48cetirizine44 28 11 2413.3RNASE3, IL8, IGHE
49estrogen4410.2CFTR, SLPI, IGHE, SERPINA3, ELANE, RNASE3
50nedocromil sodium4410.1IL8, RNASE3, IGHE

GO Terms for genes affiliated with Bronchiectasis

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16Gene Ontology
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Cellular components related to Bronchiectasis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1sodium channel complexGO:03470610.5SCNN1B, SCNN1A, SCNN1G
2apical plasma membraneGO:01632410.4CFTR, SCNN1G, SCNN1A, SCNN1B
3cell surfaceGO:00998610.3TLR2, MBL2, CTSG, CFTR, ELANE
4extracellular spaceGO:00561510.3MBL2, CTSG, MPO, IL8, MMP8, SERPINA1
5external side of plasma membraneGO:00989710.3TLR2, SCNN1B, SCNN1A, SCNN1G
6extracellular regionGO:00557610.2ELANE, BPI, MBL2, CTSG, IGHE, RNASE3
7secretory granuleGO:03014110.1ELANE, MPO, CTSG

Biological processes related to Bronchiectasis according to GeneCards/GeneDecks:

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1sensory perception of tasteGO:05090910.7SCNN1B, SCNN1A, SCNN1G
2excretionGO:00758810.7SCNN1G, SCNN1A, SCNN1B
3acute-phase responseGO:00695310.6SERPINA3, SERPINA1, MBL2
4response to yeastGO:00187810.6ELANE, MPO
5negative regulation of growth of symbiont in hostGO:04413010.6ELANE, MPO, CTSG, MBL2, TLR2
6response to stimulusGO:05089610.6SCNN1B, SCNN1A, SCNN1G
7positive regulation of immune responseGO:05077810.6CTSG, ELANE
8immune responseGO:00695510.6BPI, TLR2, CTSG, IGHE, IL8
9negative regulation of endopeptidase activityGO:01095110.5SERPINA3, SERPINA1, SLPI
10opsonizationGO:00822810.5MBL2, FCN2
11complement activation, lectin pathwayGO:00186710.5MBL2, FCN2
12sodium ion transportGO:00681410.4SCNN1B, SCNN1A, SCNN1G
13defense response to bacteriumGO:04274210.4RNASE3, MBL2, BPI
14transmembrane transportGO:05508510.3CFTR, TAP2, SCNN1G, SCNN1A, SCNN1B
15extracellular matrix disassemblyGO:02261710.1CTSG, MMP8, ELANE

Molecular functions related to Bronchiectasis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1ligand-gated sodium channel activityGO:01528010.4SCNN1G, SCNN1A, SCNN1B
2serine-type endopeptidase inhibitor activityGO:00486710.4SERPINA3, SERPINA1, SLPI
3WW domain bindingGO:05069910.3SCNN1G, SCNN1A, SCNN1B
4protein bindingGO:0055159.8SCNN1G, SCNN1A, SCNN1B, MBL2, TLR2, IL8

Products for genes affiliated with Bronchiectasis

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  • Antibodies
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  • Lysates
  • Antibodies

Sources for Bronchiectasis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet