MCID: IVM001
MIFTS: 32

Ivemark Syndrome malady

Cardiovascular diseases category

Summaries for Ivemark Syndrome

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42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 Ivemark syndrome is a rare condition that affects multiple organ systems of the body. it is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleen, heart malformations, and the abnormal arrangement of the internal organs of the chest and abdomen (situs inversus). symptoms vary greatly depending on the specific abnormalities present. the exact cause of ivemark syndrome is not known. last updated: 9/20/2011

MalaCards: Ivemark Syndrome, also known as bilateral right-sidedness sequence, is related to pancreatitis and right atrial isomerism. An important gene associated with Ivemark Syndrome is LEFTY2 (left-right determination factor 2), and among its related pathways are TGF Beta Signaling Pathway and Signaling by NODAL. The compounds 4-Carboxycinnamic Acid and 1,3,5-BENZENETRICARBOXYLIC ACID have been mentioned in the context of this disorder. Affiliated tissues include spleen, heart and kidney, and related mouse phenotype liver/biliary system.

Wikipedia:63 Asplenia with cardiovascular anomalies, also known as Ivemark syndrome and right atrial isomerism, is an... more...

Aliases & Classifications for Ivemark Syndrome

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42NIH Rare Diseases, 22GTR
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Cardiovascular diseases


Aliases & Descriptions:

ivemark syndrome 42
bilateral right-sidedness sequence 42 22
asplenia with cardiovascular anomalies 42
splenic agenesis syndrome 42
asplenia syndrome 42


Related Diseases for Ivemark Syndrome

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Ivemark Syndrome:



Diseases related to ivemark syndrome

Clinical Features for Ivemark Syndrome

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Drugs & Therapeutics for Ivemark Syndrome

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Ivemark Syndrome

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22GTR
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Genetic tests related to Ivemark Syndrome:

id Genetic test Affiliating Genes
1 Bilateral Right-Sidedness Sequence22

Anatomical Context for Ivemark Syndrome

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32MalaCards
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MalaCards organs/tissues related to Ivemark Syndrome:

32
Spleen, Heart, Kidney, Lung

Animal Models for Ivemark Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Ivemark Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.6LEFTY1, LEFTY2, HBB, MUC1

Publications for Ivemark Syndrome

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Genetic Variations for Ivemark Syndrome

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Expression for genes affiliated with Ivemark Syndrome

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

Search GEO for disease gene expression data for Ivemark Syndrome.

Pathways for genes affiliated with Ivemark Syndrome

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Sources:
37NCBI BioSystems Database, 53Reactome, 29KEGG
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Compounds for genes affiliated with Ivemark Syndrome

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11DrugBank, 44Novoseek, 24HMDB
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Compounds related to Ivemark Syndrome according to GeneCards/GeneDecks:

(show all 15)
idCompoundScoreTop Affiliating Genes
14-Carboxycinnamic Acid119.8HBA1, HBB
21,3,5-BENZENETRICARBOXYLIC ACID119.8HBB, HBA1
3SEBACIC ACID119.8HBB, HBA1
42-{4-[(3,5-DIMETHYLANILINO)-CARBONYL-METHYL]-PHENOXY}-2-METHYLPROPIONIC ACID119.8HBB, HBA1
54-[(5-methoxy-2-methylphenoxy)methyl]pyridine119.7HBB, HBA1
62,6-DICARBOXYNAPHTHALENE119.7HBB, HBA1
72-[(2-methoxy-5-methylphenoxy)methyl]pyridine119.7HBB, HBA1
82-[4-({[(3,5-DICHLOROPHENYL)AMINO]CARBONYL}AMINO)PHENOXY]-2-METHYLPROPANOIC ACID119.7HBB, HBA1
9iron dextran44 1110.6HBB, HBA1
1023-diphosphoglycerate449.4HBB, HBA2
11hmba449.4HBB, HBA2
12butyrate448.9MUC1, HBA2, HBB
13thymidine44 249.9HBB, HBA2, MUC1
14iron44 249.6HBA1, HBB, HBA2
15oxygen44 249.4MUC1, HBA2, HBB, HBA1

GO Terms for genes affiliated with Ivemark Syndrome

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

idNameGO IDScoreTop Affiliating Genes
1endocytic vesicle lumenGO:0716829.3HBB, HBA2
2haptoglobin-hemoglobin complexGO:0318389.2HBB, HBA2
3hemoglobin complexGO:0058339.0HBB, HBA2

Biological processes related to Ivemark Syndrome according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1cell growthGO:0160499.6LEFTY1, LEFTY2
2oxygen transportGO:0156719.5HBB, HBA2
3positive regulation of cell deathGO:0109429.4HBB, HBA2
4hydrogen peroxide catabolic processGO:0427449.4HBB, HBA2
5bicarbonate transportGO:0157019.3HBA2, HBB
6response to hydrogen peroxideGO:0425429.2HBB, HBA2
7protein heterooligomerizationGO:0512919.0HBB, HBA2

Molecular functions related to Ivemark Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1transforming growth factor beta receptor bindingGO:0051609.6LEFTY1, LEFTY2
2haptoglobin bindingGO:0317209.4HBB, HBA2
3oxygen bindingGO:0198259.3HBB, HBA2
4oxygen transporter activityGO:0053449.2HBA2, HBB
5peroxidase activityGO:0046019.0HBB, HBA2

Products for genes affiliated with Ivemark Syndrome

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Sources for Ivemark 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