MCID: MCR225
MIFTS: 44

Macrophage Activation Syndrome

Categories: Blood diseases, Bone diseases, Rare diseases

Aliases & Classifications for Macrophage Activation Syndrome

MalaCards integrated aliases for Macrophage Activation Syndrome:

Name: Macrophage Activation Syndrome 52 58 17 71

Classifications:

Orphanet: 58  
Rare systemic and rhumatological diseases
Rare immunological diseases


External Ids:

MESH via Orphanet 44 D055501
UMLS via Orphanet 72 C1096155
Orphanet 58 ORPHA158061
UMLS 71 C1096155

Summaries for Macrophage Activation Syndrome

MalaCards based summary : Macrophage Activation Syndrome is related to griscelli syndrome, type 2 and griscelli syndrome. An important gene associated with Macrophage Activation Syndrome is RAB27A (RAB27A, Member RAS Oncogene Family), and among its related pathways/superpathways are Innate Immune System and Akt Signaling. The drugs Pharmaceutical Solutions and polysaccharide-K have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone and liver, and related phenotypes are homeostasis/metabolism and hematopoietic system

Wikipedia : 74 Macrophage-activation syndrome is a severe, potentially life-threatening, complication of several... more...

Related Diseases for Macrophage Activation Syndrome

Diseases related to Macrophage Activation Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 301)
# Related Disease Score Top Affiliating Genes
1 griscelli syndrome, type 2 32.4 UNC13D RAB27A PRF1
2 griscelli syndrome 32.4 UNC13D RAB27A PRF1
3 adult-onset still's disease 32.0 TNFRSF1A TNF IL18
4 systemic onset juvenile idiopathic arthritis 31.3 TNF IL18
5 juvenile rheumatoid arthritis 31.1 TNFRSF1B TNFRSF1A TNF IL18
6 pancytopenia 30.6 UNC13D RAB27A PRF1
7 hemophagocytic lymphohistiocytosis, familial, 1 30.4 UNC13D RAB27A PRF1
8 primary hemophagocytic lymphohistiocytosis 30.3 UNC13D RAB27A PRF1
9 hemophagocytic lymphohistiocytosis 30.1 UNC13D TNF RAB27A PRF1 IL18
10 myocarditis 29.9 TNF PRF1 IL18
11 thrombocytopenia 29.7 TNF PRF1 IL18 ANGPT2 ANGPT1
12 lupus erythematosus 29.6 TNFRSF1B TNF IRF5 HMGB1
13 enthesopathy 29.5 TNFRSF1B TNF
14 familial mediterranean fever 29.4 TNFRSF1A TNF IL18
15 juvenile ankylosing spondylitis 29.2 TNFRSF1B TNF
16 rheumatic disease 29.2 TNFRSF1B TNF IL18 HMGB1 CD163
17 nocardiosis 29.1 TNFRSF1B TNF
18 autoimmune disease 29.1 TNFRSF1B TNF PRF1 IRF5 IL18
19 mevalonic aciduria 28.8 TNFRSF1B TNFRSF1A NLRC4
20 immune deficiency disease 28.7 TNFRSF1A TNF RAB27A IL18
21 spondyloarthropathy 1 28.7 TNFRSF1B TNFRSF1A TNF CD163
22 sarcoidosis 1 28.6 TNFRSF1B TNF IL18
23 periodic fever, familial, autosomal dominant 28.5 TNFRSF1B TNFRSF1A TNF
24 crohn's disease 28.3 TNFRSF1B TNF IRF5 IL18
25 systemic lupus erythematosus 28.3 TNFRSF1B TNFRSF1A TNF IRF5 IL18 HMGB1
26 mycobacterium tuberculosis 1 27.8 TNFRSF1B TNFRSF1A TNF PRF1 IL18
27 arthritis 27.8 TNFRSF1B TNFRSF1A TNF IRF5 IL18 HMGB1
28 rheumatoid arthritis 27.6 TNFRSF1B TNFRSF1A TNF IRF5 IL18 HMGB1
29 malaria 27.2 TNFRSF1B TNFRSF1A TNF PRF1 IL18 CD163
30 inflammatory bowel disease 26.9 TNFRSF1B TNFRSF1A TNF IRF5 IL18 HMGB1
31 autoinflammation with infantile enterocolitis 11.9
32 lipodermatosclerosis 10.4 ANGPT2 ANGPT1
33 kawasaki disease 10.4
34 malignant renovascular hypertension 10.4 ANGPT2 ANGPT1
35 exanthem 10.4
36 malignant hypertensive renal disease 10.4 ANGPT2 ANGPT1
37 anca-associated vasculitis 10.4 HMGB1 ANGPT2
38 secondary hemophagocytic lymphohistiocytosis 10.4
39 hypertriglyceridemia, familial 10.3
40 splenomegaly 10.3
41 disseminated intravascular coagulation 10.2
42 cardiac rupture 10.2 TNF PRF1
43 renal infectious disease 10.2 NLRC4 IL18
44 staphylococcal toxic shock syndrome 10.2 TNF HMGB1
45 oral tuberculosis 10.2 TNF PRF1
46 dermatomyositis 10.2
47 hemophagocytic lymphohistiocytosis, familial, 3 10.1 UNC13D RAB27A
48 cytokine deficiency 10.1
49 stachybotrys chartarum 10.1 TNF IL18
50 twin-to-twin transfusion syndrome 10.1 ANGPT2 ANGPT1

Graphical network of the top 20 diseases related to Macrophage Activation Syndrome:



Diseases related to Macrophage Activation Syndrome

Symptoms & Phenotypes for Macrophage Activation Syndrome

MGI Mouse Phenotypes related to Macrophage Activation Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.15 ANGPT1 ANGPT2 CD163 FTH1 IL18 IRF5
2 hematopoietic system MP:0005397 10.14 CD163 FTH1 IL18 IRF5 NLRC4 PRF1
3 cellular MP:0005384 10.13 ANGPT2 CD163 FTH1 IL18 NLRC4 PRF1
4 immune system MP:0005387 10.13 ANGPT1 ANGPT2 CD163 FTH1 IL18 IRF5
5 cardiovascular system MP:0005385 10.11 ANGPT1 ANGPT2 CD163 FTH1 IL18 PRF1
6 nervous system MP:0003631 9.81 ANGPT1 FTH1 IL18 PRF1 RAB27A TNF
7 integument MP:0010771 9.8 ANGPT2 IL18 PRF1 RAB27A TNF TNFRSF1A
8 respiratory system MP:0005388 9.43 ANGPT2 CD163 RAB27A TNF TNFRSF1A TNFRSF1B
9 vision/eye MP:0005391 9.23 ANGPT1 ANGPT2 CD163 IL18 RAB27A TNF

Drugs & Therapeutics for Macrophage Activation Syndrome

Drugs for Macrophage Activation Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 50)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Pharmaceutical Solutions Phase 3
2 polysaccharide-K Phase 3
3
Aldesleukin Approved Phase 1, Phase 2 110942-02-4, 85898-30-2
4
Methylprednisolone hemisuccinate Approved Phase 1, Phase 2 2921-57-5
5
Methylprednisolone Approved, Vet_approved Phase 1, Phase 2 83-43-2 6741
6
Prednisolone Approved, Vet_approved Phase 1, Phase 2 50-24-8 5755
7 Prednisolone acetate Approved, Vet_approved Phase 1, Phase 2 52-21-1
8
Prednisolone phosphate Approved, Vet_approved Phase 1, Phase 2 302-25-0
9
Melphalan Approved Phase 1, Phase 2 148-82-3 4053 460612
10
Busulfan Approved, Investigational Phase 1, Phase 2 55-98-1 2478
11
Fludarabine Approved Phase 1, Phase 2 21679-14-1, 75607-67-9 30751
12
alemtuzumab Approved, Investigational Phase 1, Phase 2 216503-57-0
13
Tacrolimus Approved, Investigational Phase 1, Phase 2 104987-11-3 445643 439492 6473866
14
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 50-18-0, 6055-19-2 2907
15
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030 46835353
16
Thiotepa Approved, Investigational Phase 1, Phase 2 52-24-4 5453
17
Prednisolone hemisuccinate Experimental Phase 1, Phase 2 2920-86-7
18 Interleukin-2 Phase 1, Phase 2
19 Analgesics, Non-Narcotic Phase 1, Phase 2
20 Anti-Retroviral Agents Phase 1, Phase 2
21 Analgesics Phase 1, Phase 2
22 Anti-HIV Agents Phase 1, Phase 2
23 Interferon-gamma Phase 2
24 interferons Phase 2
25 Anti-Infective Agents Phase 2
26 Antiviral Agents Phase 2
27 Antiemetics Phase 1, Phase 2
28 Interleukin 1 Receptor Antagonist Protein Phase 2
29 Gastrointestinal Agents Phase 1, Phase 2
30 Methylprednisolone Acetate Phase 1, Phase 2
31 Hormone Antagonists Phase 1, Phase 2
32 Neuroprotective Agents Phase 1, Phase 2
33 Autonomic Agents Phase 1, Phase 2
34 Anti-Inflammatory Agents Phase 1, Phase 2
35 Protective Agents Phase 1, Phase 2
36 Antirheumatic Agents Phase 2
37 Antineoplastic Agents, Hormonal Phase 1, Phase 2
38 glucocorticoids Phase 1, Phase 2
39 Hormones Phase 1, Phase 2
40 Alkylating Agents Phase 1, Phase 2
41 Liver Extracts Phase 1, Phase 2
42 Antineoplastic Agents, Immunological Phase 1, Phase 2
43 Immunosuppressive Agents Phase 1, Phase 2
44 Antimetabolites Phase 1, Phase 2
45 Calcineurin Inhibitors Phase 1, Phase 2
46 Immunologic Factors Phase 1, Phase 2
47 Vaccines Early Phase 1
48 Antibodies, Monoclonal
49 Immunoglobulins
50 Antibodies

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 Multicenter, Double-blind, Placebo-controlled, Randomized Withdrawal Trial With Tadekinig Alfa (r-hIL-18BP) in Patients With IL-18 Driven Monogenic Autoinflammatory Conditions: NLRC4 Mutation and XIAP Deficiency Recruiting NCT03113760 Phase 3 Tadekinig alfa
2 Safety and Efficiency Study of Low-dose IL-2 Treatment in Macrophage Activation Syndrome Unknown status NCT02569463 Phase 1, Phase 2 Interleukin-2
3 A Pilot, Open-label, Single Arm, Multicenter Study to Evaluate Safety, Tolerability, Pharmacokinetics and Efficacy of Intravenous Administrations of NI-0501, an Anti-interferon Gamma (Anti-IFNγ) Monoclonal Antibody, in Patients With Systemic Juvenile Idiopathic Arthritis (sJIA) Developing Macrophage Activation Syndrome/Secondary HLH (MAS/sHLH) Recruiting NCT03311854 Phase 2 NI-0501
4 A Personalized Randomized Trial of Validation and Restoration of Immune Dysfunction in Severe Infections and Sepsis Recruiting NCT03332225 Phase 2 Anakinra;Recombinant human interferon-gamma;Placebo
5 CD34+ Stem Cell Selection for Patients Receiving a Matched or Partially Matched Family or Unrelated Adult Donor Allogeneic Stem Cell Transplant for Malignant Disease Recruiting NCT02061800 Phase 1, Phase 2 Thiotepa;Cyclophosphamide;Alemtuzumab;Tacrolimus;Melphalan;Busulfan;Fludarabine;Methylprednisolone
6 Randomized Placebo Controlled Trial of Subcutaneous rhIL-1A (Anakinra) in the Management of Hospitalized Pediatric and Adult Patients With Macrophage Activation Syndrome Recruiting NCT02780583 Phase 1 kineret;placebo
7 Comparison of Predictive Ability of New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
8 CD34+ Stem Cell Selection for Patients Receiving a Matched or Partially Matched Family or Unrelated Adult Donor Allogeneic Stem Cell Transplantation for Non-Malignant Disease Recruiting NCT01966367 Early Phase 1
9 Investigation of the Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still s Disease and Related Inflammatory Conditions Recruiting NCT03510442
10 Retrospective Study of Immunotherapy Related Toxicities in Children and Adults With Cancer Active, not recruiting NCT03827343
11 Characterization of the Inflammatory Profile of Patients With Macrophage Activation Syndrome Secondary to Bacterial Sepsis Not yet recruiting NCT03721809
12 Single Patient Use of Tocilizumab for Treatment of Steroid Dependent, Active Systemic Onset Juvenile Idiopathic Arthritis Terminated NCT00868751

Search NIH Clinical Center for Macrophage Activation Syndrome

Genetic Tests for Macrophage Activation Syndrome

Anatomical Context for Macrophage Activation Syndrome

MalaCards organs/tissues related to Macrophage Activation Syndrome:

40
T Cells, Bone, Liver, Lung, Skin, Kidney, Monocytes

Publications for Macrophage Activation Syndrome

Articles related to Macrophage Activation Syndrome:

(show top 50) (show all 748)
# Title Authors PMID Year
1
Genetic Deficiency of Interferon-γ Reveals Interferon-γ-Independent Manifestations of Murine Hemophagocytic Lymphohistiocytosis. 61
31400073 2020
2
Adult macrophage activation syndrome-haemophagocytic lymphohistiocytosis: 'of plasma exchange and immunosuppressive escalation strategies' - a single centre reflection. 61
32013725 2020
3
Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis. 61
31707357 2020
4
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management. 61
31732958 2020
5
Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis. 61
31513353 2020
6
To "Lump" or to "Split" in Macrophage Activation Syndrome and Hemophagocytic Lymphohistiocytosis. 61
31524337 2020
7
Macrophage activation syndrome in pediatrics. 61
32017214 2020
8
IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome. 61
31326996 2020
9
IL-18 as therapeutic target in a patient with resistant systemic juvenile idiopathic arthritis and recurrent macrophage activation syndrome. 61
31359055 2020
10
Expression of concern to: High systemic levels of the cytokine-inducing HMGB1 isoform secreted in severe macrophage activation syndrome. 61
32013863 2020
11
Dysregulation of TLR9 in neonates leads to fatal inflammatory disease driven by IFN-γ. 61
31980536 2020
12
Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. 61
31924225 2020
13
Clinical characteristics and comorbidities in adult-onset Still's disease using a large US administrative claims database. 61
31965185 2020
14
Adult-onset Still's disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review. 61
31541282 2020
15
Prescribing motivations and patients' characteristics related to the use of biologic drugs in adult-onset Still's disease: analysis of a multicentre "real-life" cohort. 61
31263993 2020
16
Macrophage activation syndrome as a complication of rheumatologic disorders, a report from Iran. 61
31995957 2020
17
[Kawasaki disease with severe manifestations and macrophage activation syndrome in a pediatric patient]. 61
31758909 2019
18
Catastrophes due to missing complements: C1q deficiency lupus with Kikuchi-Fujimoto disease and macrophage activation syndrome. 61
31873753 2019
19
Outcome of children with systemic rheumatic diseases admitted to pediatric intensive care unit: An experience of a tertiary hospital. 61
31890839 2019
20
Emergent high fatality lung disease in systemic juvenile arthritis. 61
31562126 2019
21
Macrophage activation syndrome revealing colon carcinoma About a case. 61
31859642 2019
22
A rare complication of systemic lupus erythematosus in a 9-year-old girl: Questions. 61
31823041 2019
23
Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases. 61
31874111 2019
24
[Periodic fever and pancytopenia in a 35-year-old patient]. 61
31549186 2019
25
Are there new targets for juvenile idiopathic arthritis? 61
31779842 2019
26
[Clinical characteristics and treatment outcomes of macrophage activation syndrome in adults: A case series of 67 patients]. 61
31848493 2019
27
Nonspecific interstitial pneumonia in refractory systemic juvenile idiopathic arthritis responded to tocilizumab treatment. 61
31837211 2019
28
Pediatric macrophage activation syndrome, recognizing the tip of the Iceberg. 61
31804174 2019
29
Rowell syndrome complicated with macrophage activation syndrome in a child. 61
31674268 2019
30
A Multicenter Network Assessment of Three Inflammation Phenotypes in Pediatric Sepsis-Induced Multiple Organ Failure. 61
31568246 2019
31
Long-term surveillance of biologic therapies in systemic-onset juvenile idiopathic arthritis: data from the German BIKER registry. 61
31846042 2019
32
Haploidentical CD3 or α/β T-cell depleted HSCT in advanced stage sickle cell disease. 61
31089288 2019
33
Fulminant macrophage activation syndrome in a patient with anti-synthetase syndrome. 61
31774540 2019
34
Synergistic TLR/IFNα/β-Signaling Facilitates Escape of IL-18 Expression from Endotoxin Tolerance. 61
31710506 2019
35
Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report and Review of the Literature. 61
31708548 2019
36
Comparison of serum biomarkers for the diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. 61
31449879 2019
37
Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease: Characterization and Risk Factors. 61
31379071 2019
38
Predictive factors and prognosis of macrophage activation syndrome associated with adult-onset Still's disease. 61
31287402 2019
39
Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease: A case report. 61
31689747 2019
40
Extensive serum biomarker analysis in patients with macrophage activation syndrome associated with systemic lupus erythematosus. 61
31476438 2019
41
Respiratory failure and macrophage activation syndrome as an onset of systemic lupus erythematosus: A case report. 61
31799315 2019
42
Complete Atrioventricular Block and Macrophage Activation Syndrome Simultaneously Occurred in a 6-Year-Old Chinese Girl With Kawasaki Disease. 61
31743274 2019
43
Kawasaki Disease: Beyond IVIG and Aspirin. 61
31609999 2019
44
Clinical and laboratory features, treatment, and outcomes of macrophage activation syndrome in 80 children: a multi-center study in China. 61
31612427 2019
45
Severe rhabdomyolysis associated with severe fever with thrombocytopenia syndrome in a married couple: a case report. 61
31651242 2019
46
[Extra-nodal NK/T-cell lymphoma, nasal-type, revealed by cutaneous and ocular involvement]. 61
31371036 2019
47
New Onset Autoimmune Disease or Macrophage Activation Syndrome? 61
31609998 2019
48
Adapting the MS score for detection of macrophage activation syndrome in adult-onset Still's disease. Response to 'Application of MS score in macrophage activation syndrome patients associated with adult onset Still's disease' by Wang et al. 61
31653650 2019
49
Application of MS score in macrophage activation syndrome patients associated with adult onset Still's disease. 61
31611222 2019
50
Macrophage activation syndrome in neonates born to mothers with adult-onset Still's disease: Perinatal effect of maternal IL-18. 61
31299380 2019

Variations for Macrophage Activation Syndrome

Expression for Macrophage Activation Syndrome

Search GEO for disease gene expression data for Macrophage Activation Syndrome.

Pathways for Macrophage Activation Syndrome

Pathways related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

(show all 30)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.29 UNC13D TNFRSF1B TNFRSF1A TNF RAB27A NLRC4
2
Show member pathways
13.26 TNFRSF1B TNFRSF1A TNF IL18 ANGPT2 ANGPT1
3
Show member pathways
13.19 TNFRSF1B TNFRSF1A TNF IRF5 IL18 ANGPT1
4
Show member pathways
13.09 TNFRSF1B TNFRSF1A TNF IL18 ANGPT2 ANGPT1
5
Show member pathways
12.97 TNFRSF1A TNF NLRC4 IRF5 IL18
6
Show member pathways
12.56 TNFRSF1B TNFRSF1A TNF PRF1 IRF5
7
Show member pathways
12.51 TNFRSF1B TNFRSF1A TNF PRF1
8
Show member pathways
12.48 TNFRSF1B TNFRSF1A TNF IL18
9
Show member pathways
12.41 TNF IL18 ANGPT2 ANGPT1
10 12.38 TNFRSF1B TNFRSF1A TNF IRF5
11 12.15 TNFRSF1A TNF NLRC4 IL18
12
Show member pathways
12.07 TNFRSF1B TNFRSF1A TNF IL18
13
Show member pathways
12.06 TNFRSF1A TNF PRF1
14 11.83 TNFRSF1B TNF IL18
15
Show member pathways
11.82 TNFRSF1B TNFRSF1A TNF
16 11.81 TNFRSF1B TNFRSF1A TNF
17
Show member pathways
11.8 TNFRSF1A TNF HMGB1 FTH1
18 11.68 TNF IL18 ANGPT1
19
Show member pathways
11.56 TNFRSF1B TNFRSF1A TNF
20 11.54 TNF IL18 CD163
21 11.51 TNFRSF1B TNFRSF1A TNF
22 11.34 TNF NLRC4 IL18
23 11.28 TNFRSF1B TNFRSF1A TNF
24
Show member pathways
11.2 TNFRSF1B TNFRSF1A TNF
25 11.01 TNFRSF1A TNF
26 10.98 TNF IL18
27 10.84 TNFRSF1B TNFRSF1A TNF
28 10.82 UNC13D RAB27A
29 10.64 UNC13D RAB27A
30 10.61 TNFRSF1B TNFRSF1A TNF IL18

GO Terms for Macrophage Activation Syndrome

Cellular components related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane raft GO:0045121 9.56 TNFRSF1B TNFRSF1A TNF ANGPT1
2 extracellular region GO:0005576 9.4 UNC13D TNFRSF1B TNFRSF1A TNF RAB27A PRF1
3 Weibel-Palade body GO:0033093 9.16 UNC13D RAB27A
4 tumor necrosis factor receptor superfamily complex GO:0002947 8.96 TNFRSF1B TNFRSF1A

Biological processes related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

(show all 29)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of apoptotic process GO:0043065 9.92 TNF NLRC4 IRF5 HMGB1
2 neutrophil degranulation GO:0043312 9.89 UNC13D TNFRSF1B RAB27A HMGB1 FTH1
3 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.81 TNF NLRC4 IL18
4 tumor necrosis factor-mediated signaling pathway GO:0033209 9.78 TNFRSF1B TNFRSF1A TNF
5 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.76 TNF IL18 ANGPT1
6 positive regulation of inflammatory response GO:0050729 9.75 TNFRSF1A TNF IL18
7 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.73 TNFRSF1A TNF IL18
8 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.71 TNF IL18 HMGB1
9 positive regulation of exocytosis GO:0045921 9.64 UNC13D RAB27A
10 negative regulation of myoblast differentiation GO:0045662 9.64 TNF IL18
11 regulation of I-kappaB kinase/NF-kappaB signaling GO:0043122 9.63 TNF ANGPT1
12 negative regulation of cardiac muscle hypertrophy GO:0010614 9.62 TNFRSF1B TNFRSF1A
13 positive regulation of membrane protein ectodomain proteolysis GO:0051044 9.61 TNFRSF1B TNF
14 intrinsic apoptotic signaling pathway in response to DNA damage GO:0008630 9.61 TNFRSF1B TNFRSF1A TNF
15 positive regulation of neuroinflammatory response GO:0150078 9.6 TNF IL18
16 positive regulation of ceramide biosynthetic process GO:2000304 9.59 TNFRSF1A TNF
17 death-inducing signaling complex assembly GO:0071550 9.57 TNFRSF1A TNF
18 regulation of establishment of endothelial barrier GO:1903140 9.55 TNFRSF1A TNF
19 natural killer cell degranulation GO:0043320 9.52 UNC13D RAB27A
20 negative regulation of cytokine secretion involved in immune response GO:0002740 9.51 TNF ANGPT1
21 positive regulation of regulated secretory pathway GO:1903307 9.48 UNC13D RAB27A
22 aortic valve development GO:0003176 9.46 TNFRSF1B TNFRSF1A
23 glomerulus vasculature development GO:0072012 9.4 ANGPT2 ANGPT1
24 negative regulation of extracellular matrix constituent secretion GO:0003332 9.37 TNFRSF1B TNFRSF1A
25 cytokine-mediated signaling pathway GO:0019221 9.35 TNFRSF1B TNFRSF1A TNF IRF5 IL18
26 pulmonary valve development GO:0003177 9.32 TNFRSF1B TNFRSF1A
27 positive regulation of apoptotic process involved in morphogenesis GO:1902339 9.26 TNFRSF1B TNFRSF1A
28 inflammatory response GO:0006954 9.17 TNFRSF1B TNFRSF1A TNF NLRC4 IL18 HMGB1
29 Tie signaling pathway GO:0048014 9.16 ANGPT2 ANGPT1

Molecular functions related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.97 UNC13D TNFRSF1B TNFRSF1A TNF RAB27A PRF1
2 tumor necrosis factor-activated receptor activity GO:0005031 8.96 TNFRSF1B TNFRSF1A
3 tumor necrosis factor binding GO:0043120 8.62 TNFRSF1B TNFRSF1A

Sources for Macrophage Activation Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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