MCID: ACT017
MIFTS: 55

Acute Chest Syndrome malady

Summaries for Acute Chest Syndrome

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8Disease Ontology, 63Wikipedia, 32MalaCards
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Disease Ontology:8 A lung disease that involves a vasoocclusive crisis of the pulmonary vasculature seen in patients with sickle cell anemia. the crisis is initiated by a lung infection, and the resulting inflammation and loss of oxygen tension leads to sickling of red cells and further vasoocclusion. the disease has symptom fever, has symptom pleuritic chest pain, has symptom tachypnea, has symptom cough, has symptom hypoxemia, has symptom marked leukocytosis, and has symptom diffuse infiltrates on chest radiograph.

MalaCards: Acute Chest Syndrome, also known as acute chest syndrome in sickle cell disease, is related to sickle cell disease and asthma, and has symptoms including diffuse infiltrates on chest radiograph, marked leukocytosis and hypoxemia. An important gene associated with Acute Chest Syndrome is PLA2G2A (phospholipase A2, group IIA (platelets, synovial fluid)), and among its related pathways are glutamine degradation I and NO-dependent CFTR activation (normal and CF). The compounds pge2 and alpha tocopherol have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and bone marrow, and related mouse phenotypes are endocrine/exocrine gland and reproductive system.

Wikipedia:63 The acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in... more...

Aliases & Classifications for Acute Chest Syndrome

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8Disease Ontology, 10DISEASES, 44Novoseek, 60UMLS, 34MeSH, 27ICD9CM, 56SNOMED-CT
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Aliases & Descriptions:

acute chest syndrome 8 10 44 60
acute chest syndrome in sickle cell disease 8


External Ids:

Disease Ontology8 DOID:1584
MeSH34 D056586
SNOMED-CT56 372146004
ICD9CM27 517.3

Related Diseases for Acute Chest Syndrome

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

(show top 50)    (show all 106)
idRelated DiseaseScoreTop Affiliating Genes
1sickle cell disease31.6VCAM1, PLA2G10, EDN1, G6PD, NOS3, THBS1
2asthma30.7PLA2G2A, THBS1, NOS1, EDN1, VCAM1
3pneumonia30.4HMOX1, NOS1, PLA2G1B
4hypertension30.2VCAM1, NOS3, EDN1
5ischemia30.2EDN1, NOS3, NOS1, SLC12A2, HMOX1, PLA2G1B
6vascular disease30.0G6PD, VCAM1, HMOX1, NOS3, GP1BA, EDN1
7sickle cell anemia10.6
8adult syndrome10.6
9bronchitis10.2
10cerebritis10.2
11pulmonary embolism10.2
12thalassemia10.2
13acute cor pulmonale10.0
14hemoglobin d disease10.0
15lipid pneumonia10.0
16beta thalassemia10.0
17acute necrotizing encephalitis10.0
18addison's disease10.0
19aortic disease10.0
20aspiration pneumonia10.0
21encephalitis10.0
22influenza10.0
23neurologic diseases10.0
24image syndrome10.0
25antiphospholipid syndrome10.0VCAM1
26glomerulonephritis10.0VCAM1
27splenic sequestration10.0GBA2
28allergic rhinitis10.0VCAM1
29alpha thalassemia10.0G6PD
30malaria10.0G6PD
31hepatitis10.0CFB
32hypertriglyceridemia10.0VCAM1
33myositis10.0VCAM1
34uremia10.0NOS3, VCAM1
35hyperglycemia10.0VCAM1, NOS3
36hyperhomocysteinemia10.0NOS3, VCAM1
37rhinitis10.0VCAM1, PLA2G2A
38autoimmune hemolytic anemia10.0G6PD
39thromboembolism10.0NOS3, G6PD
40spinal cord ischemia10.0PLA2G2A
41glioblastoma multiforme10.0THBS1
42pancreatitis10.0PLA2G1B, PLA2G2A
43prion disease10.0HMOX1
44vitiligo10.0G6PD
45nephritis10.0VCAM1, CFB
46lupus nephritis10.0VCAM1, CFB
47sarcoidosis10.0HLA-DQB1
48essential hypertension10.0EDN1, NOS3
49prinzmetal's variant angina10.0NOS3, EDN1
50dementia10.0NOS1

Graphical network of the top 20 diseases related to Acute Chest Syndrome:



Diseases related to acute chest syndrome

Clinical Features for Acute Chest Syndrome

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8Disease Ontology
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Symptoms:

8 (show all 7)
  • diffuse infiltrates on chest radiograph
  • marked leukocytosis
  • hypoxemia
  • pleuritic chest pain
  • tachypnea
  • fever
  • cough

Drugs & Therapeutics for Acute Chest Syndrome

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

Search CenterWatch for Acute Chest Syndrome

Drug clinical trials:

Search ClinicalTrials for Acute Chest Syndrome

Search NIH Clinical Center for Acute Chest Syndrome

Search CenterWatch for Acute Chest Syndrome

Genetic Tests for Acute Chest Syndrome

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Anatomical Context for Acute Chest Syndrome

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32MalaCards
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MalaCards organs/tissues related to Acute Chest Syndrome:

32
Lung, Bone, Bone marrow, Endothelial, Kidney

Animal Models for Acute Chest Syndrome or affiliated genes

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36MGI
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Publications for Acute Chest Syndrome

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50PubMed
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Articles related to Acute Chest Syndrome:

(show top 50)    (show all 213)
idTitleAuthorsYear
1
A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan. (23553659)
2013
2
Systemic Corticosteroids in Acute Chest Syndrome: Friend or Foe? (24268617)
2013
3
Pulmonary function abnormalities and asthma are prevalent in children with sickle cell disease and are associated with acute chest syndrome. (23301782)
2013
4
Comparison of chest computed tomography features in the acute phase of cardiogenic pulmonary edema and acute respiratory distress syndrome on arrival at the emergency department. (23615573)
2013
5
A review of acute chest syndrome in pediatric sickle cell disease. (23458871)
2013
6
Acute chest syndrome of sickle cell disease. (22407374)
2012
7
Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease. (22966170)
2012
8
A fatal case of acute chest syndrome in a patient with undiagnosed sickle cell trait. (22679680)
2012
9
Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation. (22184533)
2011
10
Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. (21836136)
2011
11
The impact of parenteral narcotic choice in the development of acute chest syndrome in sickle cell disease. (21464764)
2011
12
Tapered oral dexamethasone for the acute chest syndrome of sickle cell disease. (21848879)
2011
13
Acute kidney injury in sickle patients with painful crisis or acute chest syndrome and its relation to pulmonary hypertension. (20179008)
2010
14
The impact of recurrent acute chest syndrome on the lung function of young adults with sickle cell disease. (20644948)
2010
15
Acute chest syndrome in sickle cell disease. (20575992)
2010
16
HLA DRB1*130101-DQB1*060101 haplotype is associated with acute chest syndrome in sickle cell anemia patients. (19254255)
2009
17
Urinary cysteinyl leukotriene E(4) is associated with increased risk for pain and acute chest syndrome in adults with sickle cell disease. (19127594)
2009
18
A single-institution experience with treatment of severe acute chest syndrome: lack of rebound pain with dexamethasone plus transfusion therapy. (18391705)
2008
19
Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease. (17849474)
2008
20
Temporal relationship of asthma to acute chest syndrome in sickle cell disease. (17186507)
2007
21
Addison's disease presenting as acute chest syndrome: case report and review of literature. (17111738)
2006
22
Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease. (15663785)
2005
23
Asthma in children with sickle cell disease and its association with acute chest syndrome. (15741436)
2005
24
Association of T-786C eNOS gene polymorphism with increased susceptibility to acute chest syndrome in females with sickle cell disease. (14687036)
2004
25
Safety of purified poloxamer 188 in sickle cell disease: phase I study of a non-ionic surfactant in the management of acute chest syndrome. (15182051)
2004
26
The role of secretory phospholipase A2 in acute chest syndrome. (15040432)
2004
27
Chlamydia pneumoniae and acute chest syndrome in patients with sickle cell disease. (12544773)
2003
28
Acute Chest Syndrome: the role of erythro-exchange in patients with sickle cell disease in Sicily. (12877891)
2003
29
Sickle cell patient with an acute chest syndrome and a negative chest X-ray: potential role of the ventilation and perfusion (V/Q) lung scan. (14587056)
2003
30
Mycoplasma disease and acute chest syndrome in sickle cell disease. (12837872)
2003
31
Unusual encephalopathy after acute chest syndrome in sickle cell disease: acute necrotizing encephalitis. (12368703)
2002
32
Acute chest syndrome in sickle cell disease in Saudi Arab Children in the Eastern Province. (17159388)
2002
33
Acute chest syndrome. (12370708)
2002
34
The role of rib infarcts in the acute chest syndrome of sickle cell diseases. (12673838)
2001
35
Acute chest syndrome after abdominal surgery in children with sickle cell disease: Is a laparoscopic approach better? (11329573)
2001
36
Elevated serum and bronchoalveolar lavage fluid levels of interleukin 8 and granulocyte colony-stimulating factor associated with the acute chest syndrome in patients with sickle cell disease. (11122088)
2000
37
Acute chest syndrome of sickle cell disease. (11035847)
2000
38
Acute chest syndrome in sickle cell disease. Crucial considerations in adolescents and adults. (10649675)
2000
39
Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. (10477680)
1999
40
Transgenic HbS mouse neutrophils in increased susceptibility to acute lung injury: implications for sickle acute chest syndrome. (10424612)
1999
41
Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. (9787142)
1998
42
Pulmonary function in sickle cell disease with or without acute chest syndrome. (9864008)
1998
43
Inhaled nitric oxide in sickle cell disease with acute chest syndrome. (9357905)
1997
44
Successful treatment of life-threatening acute chest syndrome of sickle cell disease with venovenous extracorporeal membrane oxygenation. (9329470)
1997
45
Successful use of extracorporeal membrane oxygenation in the treatment of acute chest syndrome in a child with severe sickle cell anemia. (8725698)
1996
46
Acute chest syndrome in children with sickle cell disease. (10829868)
1995
47
Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. (8193347)
1994
48
Albuterol inhalations in acute chest syndrome. (2035485)
1991
49
Acute chest syndrome in sickle cell disease. (3761099)
1986
50
Acute reversible pulmonary ischemia. A cause of the acute chest syndrome in sickle cell disease. (4064460)
1985

Genetic Variations for Acute Chest Syndrome

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Expression for genes affiliated with Acute Chest Syndrome

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

Search GEO for disease gene expression data for Acute Chest Syndrome.

Pathways for genes affiliated with Acute Chest Syndrome

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Sources:
37NCBI BioSystems Database, 29KEGG, 53Reactome, 12EMD Millipore, 51QIAGEN
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Pathways related to Acute Chest Syndrome according to GeneCards/GeneDecks:

(show all 15)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.0NOS3, NOS1
2
NO-dependent CFTR activation (normal and CF)
10.0NOS1, NOS3
39.8NOS3, HMOX1, EDN1
4
Hide members
9.8PLA2G1B, PLA2G2A, PLA2G10
5
Hide members
9.8PLA2G10, PLA2G2A, PLA2G1B
6
Anandamide biosynthesis and metabolism
Hide members
9.8PLA2G1B, PLA2G2A, PLA2G10
79.8PLA2G1B, PLA2G2A, PLA2G10
8
Hide members
9.8PLA2G1B, PLA2G2A, PLA2G10
9
Hide members
9.7PLA2G10, PLA2G2A, PLA2G1B, NOS3
10
Hide members
9.6GP1BA, THBS1, NOS3, NOS1
11
Hide members
9.6PLA2G1B, PLA2G2A, PLA2G10, HLA-DQB1
129.5SLC12A2, PLA2G1B, PLA2G2A, PLA2G10
13
Hide members
9.4HMOX1, SLC12A2, NOS3, NOS1
14
Hide members
9.4PLA2G10, PLA2G2A, PLA2G1B, NOS3, NOS1
15
Hide members
8.9NOS1, GBA2, PLA2G10, PLA2G2A, PLA2G1B, HMOX1

Compounds for genes affiliated with Acute Chest Syndrome

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Sources:
44Novoseek, 11DrugBank, 49PharmGKB, 59Tocris Bioscience, 28IUPHAR, 24HMDB, 2BitterDB
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Compounds related to Acute Chest Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 128)
idCompoundScoreTop Affiliating Genes
1pge24410.7PLA2G10
2alpha tocopherol4410.4PLA2G2A, G6PD
3N-{(4s)-4-Amino-5-[(2-Aminoethyl)Amino]Pentyl}-N'-Nitroguanidine1110.3NOS3, NOS1
4sn mesoporphyrin4410.3G6PD, HMOX1
5N-{(3R,4S)-4-[(6-amino-4-methylpyridin-2-yl)methyl]pyrrolidin-3-yl}-N'-(3-chlorobenzyl)ethane-1,2-diamine1110.3NOS3, NOS1
6Nitroarginine1110.3NOS1, NOS3
7N-{(3S,4S)-4-[(6-AMINO-4-METHYLPYRIDIN-2-YL)METHYL]PYRROLIDIN-3-YL}-N'-(4-CHLOROBENZYL)ETHANE-1,2-DIAMINE1110.3NOS3, NOS1
8isosorbide dinitrate44 49 1112.2NOS3, HMOX1, EDN1
9L-N(Omega)-Nitroarginine-(4r)-Amino-L-Proline Amide1110.2NOS3, NOS1
10S-Ethyl-N-Phenyl-Isothiourea1110.2NOS3, NOS1
11diphenyleneiodonium4410.2VCAM1, PLA2G2A, NOS3, G6PD
12ptio4410.2NOS1, HMOX1, NOS3
13L-N(Omega)-Nitroarginine-2,4-L-Diaminobutyric Amide1110.2NOS3, NOS1
14adma4410.2NOS1, EDN1, NOS3
15n(g)-nitro-l-arginine methyl ester4410.1NOS3, EDN1, NOS1
16ng-nitro-l-arginine4410.1EDN1, NOS3, NOS1
17zinc protoporphyrin4410.1G6PD, HMOX1, NOS1
188-isoprostane4410.1EDN1, HMOX1, VCAM1, NOS3
19apocynin44 5911.1VCAM1, NOS3, NOS1, EDN1
20rottlerin4410.1EDN1, VCAM1, NOS3, HMOX1
21l-nmma4410.0NOS1, EDN1, VCAM1, NOS3
22s-nitroso-n-acetylpenicillamine4410.0NOS1, NOS3, EDN1, HMOX1
23pdtc4410.0VCAM1, NOS1, HMOX1, PLA2G2A
24l-arginine28 11 2412.0ART4, NOS1, NOS3
25nadph44 2410.9PLA2G1B, NOS1, PLA2G2A, G6PD
26alpha-linolenic acid44 11 2411.9PLA2G10, PLA2G2A, PLA2G1B
27phosphatidylserine44 28 1111.9THBS1, PLA2G2A, PLA2G10, GP1BA, G6PD
28fmlp449.8PLA2G10, PLA2G2A, PLA2G1B, THBS1, VCAM1
29txb2449.8PLA2G1B, EDN1, PLA2G10, PLA2G2A, THBS1
30phosphatidylcholine449.8G6PD, PLA2G10, PLA2G2A, PLA2G1B, VCAM1
31aspirin44 49 28 2412.8PLA2G2A, G6PD, GP1BA, NOS3, HMOX1
32atorvastatin44 49 28 11 2413.8EDN1, PLA2G2A, HMOX1, THBS1, NOS3, VCAM1
33tamoxifen44 49 28 1112.7VCAM1, NOS3, THBS1, G6PD, NOS1
34ascorbic acid44 2410.7PLA2G2A, G6PD, NOS3, HMOX1, NOS1
35rosiglitazone44 49 28 11 2413.7HMOX1, THBS1, NOS3, VCAM1
36ketamine44 28 1111.7HMOX1, NOS3, NOS1
37genistein44 28 59 2 11 2414.7VCAM1, NOS3, EDN1, NOS1, PLA2G2A, THBS1
38sodium nitroprusside449.6VCAM1, HMOX1, NOS1, EDN1, G6PD, NOS3
39glycerol44 11 2411.6PLA2G10, PLA2G2A, PLA2G1B, NOS3, NOS1, G6PD
40sb 20358044 5910.6EDN1, THBS1, NOS3, NOS1, VCAM1, HMOX1
41prostacyclin449.5EDN1, PLA2G2A, GP1BA, VCAM1, NOS3, THBS1
42lactate449.5PLA2G1B, G6PD, GP1BA, PLA2G2A, HMOX1, VCAM1
43acetylcholine44 49 28 11 2413.4EDN1, PLA2G2A, NOS3, HMOX1, NOS1
44oxygen44 2410.4CYGB, HMOX1, SLC12A2, NOS1, PLA2G1B
45vegf449.4PLA2G2A, NOS1, NOS3, HMOX1, VCAM1, EDN1
46indomethacin44 59 28 1112.3NOS3, EDN1, GP1BA, NOS1, HMOX1, PLA2G2A
47superoxide44 2410.2G6PD, PLA2G1B, HMOX1, PLA2G2A, NOS3, NOS1
48histamine44 28 2411.1EDN1, VCAM1, HMOX1, PLA2G2A, NOS1
49h2o2449.0CYGB, NOS1, THBS1, HMOX1, PLA2G1B, PLA2G2A
50nitric oxide44 11 2410.9CYGB, HMOX1, EDN1, THBS1, NOS3, VCAM1

GO Terms for genes affiliated with Acute Chest Syndrome

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16Gene Ontology
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Cellular components related to Acute Chest Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.5THBS1, PLA2G1B, PLA2G2A
2extracellular spaceGO:0056159.1VCAM1, THBS1, HMOX1, PLA2G1B, PLA2G2A, EDN1

Biological processes related to Acute Chest Syndrome according to GeneCards/GeneDecks:

(show all 22)
idNameGO IDScoreTop Affiliating Genes
1smooth muscle hyperplasiaGO:01480610.4NOS3, HMOX1
2regulation of systemic arterial blood pressure by endothelinGO:00310010.3EDN1, NOS3
3positive regulation of guanylate cyclase activityGO:03128410.3NOS1, NOS3
4chronic inflammatory responseGO:00254410.3VCAM1, THBS1
5negative regulation of hydrolase activityGO:05134610.3NOS3, NOS1
6arginine catabolic processGO:00652710.3NOS1, NOS3
7negative regulation of extrinsic apoptotic signaling pathway via death domain receptorsGO:190204210.2NOS3, HMOX1
8phosphatidylinositol acyl-chain remodelingGO:03614910.2PLA2G1B, PLA2G2A, PLA2G10
9phosphatidylserine acyl-chain remodelingGO:03615010.2PLA2G10, PLA2G2A, PLA2G1B
10phosphatidylglycerol acyl-chain remodelingGO:03614810.2PLA2G10, PLA2G2A, PLA2G1B
11phosphatidylethanolamine acyl-chain remodelingGO:03615210.2PLA2G10, PLA2G2A, PLA2G1B
12positive regulation of macrophage derived foam cell differentiationGO:01074410.1PLA2G2A, PLA2G10
13negative regulation of potassium ion transportGO:04326710.1NOS3, NOS1
14phosphatidylcholine acyl-chain remodelingGO:03615110.1PLA2G10, PLA2G2A, PLA2G1B
15phosphatidic acid biosynthetic processGO:00665410.1PLA2G1B, PLA2G2A, PLA2G10
16positive regulation of vasodilationGO:04590910.1NOS1, NOS3, HMOX1
17nitric oxide biosynthetic processGO:0068099.9NOS3, NOS1
18positive regulation of angiogenesisGO:0457669.9HMOX1, THBS1, NOS3
19response to hypoxiaGO:0016669.9EDN1, THBS1, NOS1, VCAM1
20glycerophospholipid biosynthetic processGO:0464749.8PLA2G1B, PLA2G2A, PLA2G10
21intracellular protein kinase cascadeGO:0072439.4HMOX1, PLA2G1B, EDN1
22small molecule metabolic processGO:0442819.2G6PD, GBA2, PLA2G10, PLA2G2A, PLA2G1B, HMOX1

Molecular functions related to Acute Chest Syndrome according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1nitric-oxide synthase activityGO:00451710.2NOS1, NOS3
2arginine bindingGO:03461810.1NOS3, NOS1
3tetrahydrobiopterin bindingGO:03461710.1NOS3, NOS1
4phospholipase A2 activityGO:00462310.0PLA2G1B, PLA2G2A, PLA2G10
5NADPH-hemoprotein reductase activityGO:00395810.0NOS1, NOS3
6FMN bindingGO:01018110.0NOS1, NOS3
7NADP bindingGO:05066110.0G6PD, NOS3, NOS1
8calcium-dependent phospholipase A2 activityGO:0474989.9PLA2G2A, PLA2G1B
9cadmium ion bindingGO:0468709.7NOS3, NOS1
10heme bindingGO:0200379.7NOS1, NOS3, HMOX1, CYGB

Products for genes affiliated with Acute Chest Syndrome

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Acute Chest Syndrome

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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