LVNC
MCID: LFT003
MIFTS: 37

Left Ventricular Noncompaction (LVNC) malady

Summaries for Left Ventricular Noncompaction

About this section
Sources:
42NIH Rare Diseases, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NIH Rare Diseases:42 Left ventricular noncompaction (lvnc) is a rare heart condition. in lvnc the inside wall of the heart is spongy or grooved, instead of smooth. signs and symptoms of lvnc vary, but may cause life-threatening abnormal heart rhythms and weakness of the heart muscle. treatments, such as blood thinning medication and defibrillators, are available to control these heart symptoms. in rare cases, heart transplantation is needed. last updated: 12/29/2013

MalaCards: Left Ventricular Noncompaction, also known as left ventricular hypertrabeculation, is related to myopathy and barth syndrome. An important gene associated with Left Ventricular Noncompaction is TAZ (tafazzin), and among its related pathways are Cardiac Progenitor Differentiation and Muscular Dystrophies and Dystrophin-Glycoprotein Complex. The compounds valine and ruthenium have been mentioned in the context of this disorder. Affiliated tissues include heart, and related mouse phenotypes are behavior/neurological and normal.

Aliases & Classifications for Left Ventricular Noncompaction

About this section
Sources:
42NIH Rare Diseases, 22GTR, 60UMLS
See all sources

Aliases & Descriptions:

left ventricular noncompaction 42 22 60
left ventricular hypertrabeculation 42
spongy myocardium 42
lvnc 42


Related Diseases for Left Ventricular Noncompaction

About this section
Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Left Ventricular Noncompaction 6 family:

left ventricular noncompaction Left Ventricular Noncompaction 5
Left Ventricular Noncompaction 4 Left Ventricular Noncompaction 8
Left Ventricular Noncompaction 2 Left Ventricular Noncompaction 10
Left Ventricular Noncompaction 9 Left Ventricular Noncompaction 7

Diseases related to Left Ventricular Noncompaction via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 77)
idRelated DiseaseScoreTop Affiliating Genes
1myopathy30.9LDB3, DTNA, MYH7, TAZ, LMNA, TPM1
2barth syndrome30.9DTNA, TAZ, LDB3
3hypertrophic cardiomyopathy30.9MYBPC3, MYH7
4left ventricular noncompaction 630.7TNNT2, CMD1D
5dilated cardiomyopathy30.7MYBPC3, MYH7, RYR2, TAZ, LMNA, ACTC1
6myotonic dystrophy30.5MYH7
7myocardial infarction30.5TNNT2, LDB3
8duchenne muscular dystrophy30.5SNTA1, DTNB
9long qt syndrome30.5RYR2, MYH7, SNTA1, SCN5A
10familial hypertrophic cardiomyopathy30.5ACTC1, CSRP3, TNNT2, MYBPC3, MYH7, TPM1
11sudden infant death syndrome30.3SCN5A
12congestive heart failure30.3TNNT2
13atrioventricular block30.2LMNA, SCN5A
14left ventricular noncompaction 1, with or without congenital heart defects10.6
15left ventricular noncompaction 510.5
16left ventricular noncompaction 410.5
17peripartum cardiomyopathy10.5
18left ventricular noncompaction 210.4
19myotonic dystrophy type 110.4
20ebstein anomaly10.3
21sotos syndrome10.3
22sensorineural hearing loss10.3
23brugada syndrome10.3
24hypothyroidism10.3
25muscular dystrophy10.3
26ventricular septal defect10.3
27left ventricular noncompaction 810.3
28left ventricular noncompaction 3, with or without dilated cardiomyopathy10.3
29left ventricular noncompaction 1010.3
30left ventricular noncompaction 910.3
31left ventricular noncompaction 710.3
32inclusion body myositis10.2
33adult syndrome10.2
34charcot-marie-tooth disease10.2
35myositis10.2
36poliomyelitis10.2
37tooth disease10.2
38toxoplasmosis10.2
39lown-ganong-levine syndrome10.2
40marfan syndrome10.2
41mitral valve prolapse10.2
42periventricular nodular heterotopia10.2
43pancytopenia10.2
44endomyocardial fibrosis10.2
45neuronitis10.2
46becker muscular dystrophy10.2
47tetralogy of fallot10.2
48fabry disease10.2
49coffin-lowry syndrome10.2
50patent ductus arteriosus10.2

Graphical network of the top 20 diseases related to Left Ventricular Noncompaction:



Diseases related to left ventricular noncompaction

Clinical Features for Left Ventricular Noncompaction

About this section

Drugs & Therapeutics for Left Ventricular Noncompaction

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Left Ventricular Noncompaction

Drug clinical trials:

Search ClinicalTrials for Left Ventricular Noncompaction

Search NIH Clinical Center for Left Ventricular Noncompaction

Search CenterWatch for Left Ventricular Noncompaction

Genetic Tests for Left Ventricular Noncompaction

About this section
Sources:
22GTR
See all sources

Genetic tests related to Left Ventricular Noncompaction:

id Genetic test Affiliating Genes
1 Left Ventricular Noncompaction22

Anatomical Context for Left Ventricular Noncompaction

About this section
Sources:
32MalaCards
See all sources

MalaCards organs/tissues related to Left Ventricular Noncompaction:

32
Heart

Animal Models for Left Ventricular Noncompaction or affiliated genes

About this section
Sources:
36MGI
See all sources

MGI Mouse Phenotypes related to Left Ventricular Noncompaction:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053868.4DTNA, SCN5A, SNTA1, MYBPC3, RYR2, LMNA
2MP:00028737.9DTNB, TNNT2, SCN5A, FKBP1A, RYR2, LMNA
3MP:00053787.4DTNA, CSRP3, TNNT2, SCN5A, FKBP1A, MYBPC3
4MP:00053767.0SNTA1, TNNT2, CSRP3, MBL2, FKBP1B, FKBP1A
5MP:00053696.9LDB3, DTNA, CSRP3, TNNT2, SCN5A, SNTA1
6MP:00053856.9FKBP1B, SCN5A, TNNT2, CSRP3, DTNA, FKBP1A
7MP:00107686.7DTNA, CSRP3, TNNT2, SCN5A, FKBP1B, FKBP1A

Publications for Left Ventricular Noncompaction

About this section

Genetic Variations for Left Ventricular Noncompaction

About this section

Expression for genes affiliated with Left Ventricular Noncompaction

About this section
Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Left Ventricular Noncompaction

Search GEO for disease gene expression data for Left Ventricular Noncompaction.

Pathways for genes affiliated with Left Ventricular Noncompaction

About this section
Sources:
37NCBI BioSystems Database, 51QIAGEN, 53Reactome, 4Cell Signaling Technology, 29KEGG, 49PharmGKB
See all sources

Compounds for genes affiliated with Left Ventricular Noncompaction

About this section
Sources:
44Novoseek, 24HMDB, 28IUPHAR, 59Tocris Bioscience, 49PharmGKB, 2BitterDB, 11DrugBank
See all sources

Compounds related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1valine449.6MYH7, MT-ND1, SCN5A, MBL2
2ruthenium449.4RYR2, FKBP1A
3creatinine449.3MBL2, TNNT2, MYH7, LMNA, ACTC1
4sodium44 2410.2MBL2, SCN5A, SNTA1, MYH7, RYR2
5ryanodine44 28 5911.0FKBP1B, FKBP1A, MYH7, RYR2
6arginine448.8LMNA, MYH7, MYBPC3, FKBP1A, SCN5A
7caffeine44 49 28 2 11 2413.7FKBP1B, FKBP1A, RYR2
8atp44 289.3MBL2, MT-ND1, FKBP1A, MYBPC3, MYH7, RYR2
9lipid448.1MBL2, FKBP1B, FKBP1A, MYH7, RYR2, TAZ
10calcium44 49 11 2410.3TPM1, MBL2, TNNT2, SCN5A, SNTA1, FKBP1B

GO Terms for genes affiliated with Left Ventricular Noncompaction

About this section
Sources:
16Gene Ontology
See all sources

Cellular components related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1calcium channel complexGO:0347049.6RYR2, FKBP1B
2sarcomereGO:0300179.4TPM1, ACTC1, MYH7, MYBPC3, TNNT2
3cytoskeletonGO:0058569.1CSRP3, SNTA1, LDB3, TPM1
4sarcoplasmic reticulum membraneGO:0330178.9RYR2, FKBP1A, FKBP1B
5Z discGO:0300188.2CSRP3, FKBP1B, FKBP1A, MYH7, RYR2, LDB3

Biological processes related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

(show all 31)
idNameGO IDScoreTop Affiliating Genes
1regulation of sodium ion transmembrane transportGO:190230510.3SCN5A, SNTA1
2positive regulation of ATPase activityGO:03278110.2TNNT2, MYBPC3, TPM1
3ATP catabolic processGO:00620010.1ACTC1, MYH7, TNNT2
4cardiac myofibril assemblyGO:05500310.1ACTC1, CSRP3
5cardiac muscle tissue developmentGO:04873810.1CSRP3, TAZ
6regulation of muscle contractionGO:00693710.1TNNT2, TPM1
7cardiac muscle hypertrophyGO:00330010.1RYR2, CSRP3
8regulation of ventricular cardiac muscle cell membrane repolarizationGO:06030710.1SCN5A, SNTA1
9regulation of ventricular cardiac muscle cell action potentialGO:08600510.0RYR2, SNTA1, SCN5A
10sarcomere organizationGO:04521410.0TPM1, LDB3, MYBPC3, TNNT2
11positive regulation of sequestering of calcium ionGO:05128410.0RYR2, FKBP1B
12release of sequestered calcium ion into cytosol by sarcoplasmic reticulumGO:0148089.9FKBP1B, RYR2
13cell communication by electrical coupling involved in cardiac conductionGO:0860649.9FKBP1B, RYR2
14response to redox stateGO:0517759.9FKBP1B, RYR2
15calcium-mediated signaling using intracellular calcium sourceGO:0355849.8FKBP1B, RYR2
16muscle filament slidingGO:0300499.8TNNT2, MYBPC3, MYH7, ACTC1, TPM1
17negative regulation of release of sequestered calcium ion into cytosolGO:0512809.7FKBP1B, FKBP1A
18negative regulation of protein phosphatase type 2B activityGO:0325139.7FKBP1B, FKBP1A
19de novo protein foldingGO:0064589.7FKBP1B, FKBP1A
20protein maturation by protein foldingGO:0224179.6FKBP1B, FKBP1A
21protein refoldingGO:0420269.6FKBP1B, FKBP1A
22negative regulation of ryanodine-sensitive calcium-release channel activityGO:0603159.6FKBP1B, FKBP1A
23regulation of heart rateGO:0020279.5SCN5A, SNTA1, MYBPC3, MYH7, RYR2
24regulation of ryanodine-sensitive calcium-release channel activityGO:0603149.5FKBP1B, FKBP1A
25cytosolic calcium ion homeostasisGO:0514809.5FKBP1B, RYR2
26muscle contractionGO:0069369.5SNTA1, FKBP1B, MYH7, TAZ, TPM1
27ventricular cardiac muscle tissue morphogenesisGO:0550109.3TPM1, MYH7, MYBPC3, FKBP1A, TNNT2
28peptidyl-proline modificationGO:0182089.3FKBP1A, FKBP1B
29protein peptidyl-prolyl isomerizationGO:0004139.3FKBP1A, FKBP1B
30regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ionGO:0108819.3FKBP1B, RYR2
31cardiac muscle contractionGO:0600489.0TPM1, ACTC1, TAZ, RYR2, MYBPC3, SCN5A

Molecular functions related to Left Ventricular Noncompaction according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1nitric-oxide synthase bindingGO:05099810.0SCN5A, SNTA1
2myosin bindingGO:01702210.0ACTC1, MYBPC3
3structural constituent of muscleGO:0083079.6TPM1, MYH7, MYBPC3, CSRP3
4ATPase activityGO:0168879.6ACTC1, MYH7, TNNT2
5calmodulin bindingGO:0055169.5RYR2, MYH7, SNTA1, SCN5A
6actin bindingGO:0037799.5TNNT2, SNTA1, MYBPC3, MYH7, TPM1
7calcium channel inhibitor activityGO:0198559.3FKBP1B, FKBP1A
8FK506 bindingGO:0055289.2FKBP1A, FKBP1B
9ryanodine-sensitive calcium-release channel activityGO:0052199.2FKBP1B, FKBP1A, RYR2
10ion channel bindingGO:0443258.7RYR2, FKBP1A, FKBP1B, SNTA1, SCN5A

Products for genes affiliated with Left Ventricular Noncompaction

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Left Ventricular Noncompaction

About this section
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