MCID: PLM006
MIFTS: 47

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 niemann-pick disease, and has symptoms including hemoptysisand hemoptysis. 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 liver, 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 106)
idRelated DiseaseScoreTop Affiliating Genes
1respiratory failure31.1SFTPB, SFTPC
2niemann-pick disease30.6SFTPB, SFTPC
3myelodysplastic syndrome30.2CSF1, CSF2, GATA2
4acquired pulmonary alveolar proteinosis12.6
5congenital pulmonary alveolar proteinosis12.5
6hereditary pulmonary alveolar proteinosis12.4
7secondary pulmonary alveolar proteinosis12.4
8severe early-onset pulmonary alveolar proteinosis due to mars deficiency12.3
9interstitial lung and liver disease11.6
10surfactant metabolism dysfunction, pulmonary, 111.5
11surfactant metabolism dysfunction, pulmonary, 511.5
12surfactant metabolism dysfunction, pulmonary, 411.5
13pulmonary surfactant metabolism dysfunction11.4
14surfactant metabolism dysfunction, pulmonary, 211.2
15surfactant metabolism dysfunction, pulmonary, 311.2
16abca3-related pulmonary surfactant metabolism dysfunction11.2
17csf2ra-related pulmonary surfactant metabolism dysfunction11.2
18sftpb-related pulmonary surfactant metabolism dysfunction11.2
19sftpc-related pulmonary surfactant metabolism dysfunction11.2
20lysinuric protein intolerance10.8
21congenital lactic acidosis, saguenay-lac-saint-jean type10.5SFTPB, SFTPC
22transcobalamin deficiency10.5SFTPB, SFTPC
23leukemia10.5
24esophageal atresia10.4SFTPB, SFTPC
25pneumonia10.4
26acute zonal occult outer retinopathy10.4SFTPB, SFTPC
27immunodeficiency 2110.4
28lung cancer10.3
29pulmonary fibrosis10.3
30tuberculosis10.3
31lung disease10.1
32myeloid leukemia10.1
33retroperitoneal germ cell neoplasm10.1CSF2, SFTPD
34disseminated eosinophilic collagen disease10.1CSF2, SFTPD
35hartnup disorder10.1SFTPB, SFTPC
36myelofibrosis10.1CSF2, CSF2RA
37systemic lupus erythematosus10.1
38aspergillosis10.1
39arthritis10.1
40silicosis10.1
41desquamative interstitial pneumonia10.1
42cryptococcosis10.1
43pneumothorax10.1
44lung squamous cell carcinoma10.1
45mediastinitis10.1
46lupus erythematosus10.1
47psoriasis10.1
48amyloidosis10.1
49kidney sarcoma10.0SFTPB, SFTPC, SFTPD
50band keratopathy10.0SFTPB, SFTPC, SFTPD

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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UMLS symptoms related to Pulmonary Alveolar Proteinosis:


hemoptysis

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, Liver, Kidney, T cells, Thyroid, Prostate

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 578)
idTitleAuthorsYear
1
Lung Transplant Recipient with Pulmonary Alveolar Proteinosis. (27213073)
2016
2
Induced sputum deposition improves diagnostic yields of pulmonary alveolar proteinosis: A clinicopathological and methodological study of 17 cases. (26583435)
2016
3
Localized Airspace Consolidation of Pulmonary Alveolar Proteinosis Mimicking Malignant Lesions in 18F-FDG PET/CT Imaging: One Case Report. (26252337)
2015
4
Pulmonary Alveolar Proteinosis: A Rare Cause of Respiratory Failure. (26208564)
2015
5
Secondary pulmonary alveolar proteinosis in hematologic malignancies. (25300566)
2014
6
Localized pulmonary alveolar proteinosis: two case reports. (25337424)
2014
7
A crazy cause of dyspnoea: pulmonary alveolar proteinosis. (25152273)
2014
8
Development of pulmonary alveolar proteinosis following exposure to dust after the Great East Japan Earthquake. (24238228)
2013
9
De novo complex X chromosome rearrangement unmasking maternally inherited CSF2RA deletion in a girl with pulmonary alveolar proteinosis. (23918747)
2013
10
Pulmonary alveolar proteinosis requiring "hybrid" extracorporeal life support, and complicated by acute necrotizing pneumonia. (23523160)
2013
11
Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis. (24275678)
2013
12
Extracorporeal membrane oxygenation to support whole-lung lavage in pulmonary alveolar proteinosis: salvage of the drowned lungs. (23328142)
2013
13
Pulmonary alveolar proteinosis: quantitative CT and pulmonary functional correlations. (21621361)
2012
14
Spontaneous pulmonary alveolar proteinosis in captive "moustached tamarins" (Saguinus mystax). (21734058)
2012
15
Pulmonary alveolar proteinosis in an indium-processing worker. (20529594)
2010
16
Pulmonary alveolar proteinosis in a 67-year-old woman with Wegener's granulomatosis. (20623200)
2010
17
Patient-derived granulocyte/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates. (20224064)
2010
18
Whole-lung lavage in infants and children with pulmonary alveolar proteinosis. (21199121)
2010
19
F-18 FDG PET/CT in pulmonary alveolar proteinosis. (19352264)
2009
20
Differences in the immunolocalization of surfactant protein (SP)-A, SP-D, and KL-6 in pulmonary alveolar proteinosis. (18251786)
2008
21
Secondary pulmonary alveolar proteinosis in a patient with chronic myeloid leukemia in the accelerated phase. (21318986)
2008
22
GM-CSF autoantibodies in pulmonary alveolar proteinosis. (17506160)
2007
23
Pulmonary alveolar proteinosis--is host defense awry? (17287475)
2007
24
Pulmonary alveolar proteinosis. (16916009)
2006
25
Pulmonary alveolar proteinosis with myeloproliferative syndrome with myelodysplasia: bronchoalveolar lavage reduces white blood cell count. (16906593)
2006
26
Serum antibody against granulocyte/macrophage colony-stimulating factor and KL-6 in idiopathic pulmonary alveolar proteinosis. (16565598)
2006
27
Pulmonary alveolar proteinosis. What is the role of GM-CSF in disease pathogenesis and treatment? (15350161)
2004
28
Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome. (12636240)
2003
29
Detection of granulocyte-macrophage colony-stimulating factor in patients with pulmonary alveolar proteinosis. (10764326)
2000
30
Pulmonary alveolar proteinosis in a child: HRCT findings before and after bronchoalveolar lavage. (9866787)
1998
31
Serum KL-6 level as a monitoring marker in a patient with pulmonary alveolar proteinosis. (10319067)
1998
32
Pulmonary alveolar proteinosis with respiratory failure--therapeutic role of bronchoscopic and whole lung lavage. (11229289)
1998
33
Immunoglobulin G is associated with surfactant protein A aggregate isolated from patients with pulmonary alveolar proteinosis. (9154892)
1997
34
Pulmonary alveolar proteinosis. (9157392)
1997
35
Interstitial lattice shadow and mediastinal lymphadenopathy with an elevation of carcinoembryonic antigen in severe pulmonary alveolar proteinosis. (1611200)
1992
36
Pulmonary alveolar proteinosis (with review of literature). (3060464)
1988
37
Pulmonary alveolar proteinosis complicated by cerebral abscess: report of a case. (3528083)
1986
38
Pulmonary alveolar proteinosis in the newborn. (3838881)
1985
39
Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years. (6492867)
1984
40
Progressive intrapartum respiratory insufficiency due to pulmonary alveolar proteinosis. Amelioration by therapeutic whole-lung bronchopulmonary lavage. (6468015)
1984
41
The immunoinhibitory activities of the lung lavage materials and sera from patients with pulmonary alveolar proteinosis (PAP). (6894313)
1981
42
Particulate concentrations in pulmonary alveolar proteinosis. (318522)
1978
43
Pulmonary Alveolar proteinosis. Lobar localization and surgical excision. (1262145)
1976
44
Pulmonary alveolar proteinosis--physiologic observations during and after pulmonary lavage. (4531921)
1973
45
Focal pulmonary alveolar proteinosis in pulmonary tuberculosis. (5766260)
1969
46
Pulmonary alveolar proteinosis. (6071228)
1967
47
Pulmonary alveolar proteinosis. Treatment in a case complicated by tuberculosis. (6066626)
1967
48
Studies of pulmonary alveolar microlithiasis and pulmonary alveolar proteinosis. (13775542)
1960
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