KEGG   PATHWAY: dsp05414
Entry
dsp05414                    Pathway                                
Name
Dilated cardiomyopathy - Dipodomys spectabilis (banner-tailed kangaroo rat)
Description
Dilated cardiomyopathy (DCM) is a heart muscle disease characterised by dilation and impaired contraction of the left or both ventricles that results in progressive heart failure and sudden cardiac death from ventricular arrhythmia. Genetically inherited forms of DCM ("familial" DCM) have been identified in 25-35% of patients presenting with this disease, and the inherited gene defects are an important cause of "familial" DCM. The pathophysiology may be separated into two categories: defects in force generation and defects in force transmission. In cases where an underlying pathology cannot be identified, the patient is diagnosed with an "idiopathic" DCM. Current hypotheses regarding causes of "idiopathic" DCM focus on myocarditis induced by enterovirus and subsequent autoimmune myocardium impairments. Antibodies to the beta1-adrenergic receptor (beta1AR), which are detected in a substantial number of patients with "idiopathic" DCM, may increase the concentration of intracellular cAMP and intracellular Ca2+, a condition often leading to a transient hyper-performance of the heart followed by depressed heart function and heart failure.
Class
Human Diseases; Cardiovascular disease
Pathway map
dsp05414  Dilated cardiomyopathy
dsp05414

Organism
Dipodomys spectabilis (banner-tailed kangaroo rat) [GN:dsp]
Gene
122116967  Itga1; integrin alpha-1 isoform X1 [KO:K06480]
122116951  Itga2; integrin alpha-2 [KO:K06481]
122100605  Itga2b; integrin alpha-IIb isoform X1 [KO:K06476]
122103268  Itga3; integrin alpha-3 [KO:K06482]
122114964  Itga4; integrin alpha-4 [KO:K06483]
122124347  Itga5; integrin alpha-5 [KO:K06484]
122120069  Itga6; integrin alpha-6 isoform X1 [KO:K06485]
122124386  integrin alpha-7 [KO:K06583]
122113136  Itga8; integrin alpha-8 [KO:K06584]
122096773  Itga9; integrin alpha-9 [KO:K06585]
122109053  Itga10; integrin alpha-10 isoform X1 [KO:K06586]
122122943  Itga11; integrin alpha-11 [KO:K06587]
122114967  Itgav; integrin alpha-V [KO:K06487]
122112991  Itgb1; integrin beta-1 [KO:K05719]
122103395  Itgb3; integrin beta-3 [KO:K06493]
122094922  Itgb4; integrin beta-4 [KO:K06525]
122097598  Itgb5; integrin beta-5 [KO:K06588]
122110843  Itgb6; integrin beta-6 [KO:K06589]
122124271  Itgb7; integrin beta-7 [KO:K06590]
122121557  Itgb8; integrin beta-8 isoform X1 [KO:K06591]
122100380  Sgca; alpha-sarcoglycan [KO:K12565]
122106663  Sgcb; beta-sarcoglycan [KO:K12566]
122112801  Sgcg; gamma-sarcoglycan [KO:K12564]
122101054  Sgcd; delta-sarcoglycan [KO:K12563]
122111925  Sgce; epsilon-sarcoglycan isoform X1 [KO:K27061]
122127041  Sgcz; zeta-sarcoglycan isoform X1 [KO:K27062]
122121860  Lama2; laminin subunit alpha-2 [KO:K05637]
122124478  Lama1; laminin subunit alpha-1 [KO:K05637]
122095549  Dag1; dystroglycan [KO:K06265]
122108190  Cav3; caveolin-3 [KO:K12959]
122099033  Nos1; nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
122120459  Snta1; alpha-1-syntrophin [KO:K24063]
122102442  Sntb1; beta-1-syntrophin [KO:K24064]
122126199  Sntb2; beta-2-syntrophin [KO:K24064]
122104295  Dtna; LOW QUALITY PROTEIN: dystrobrevin alpha [KO:K26998]
122118590  Dmd; dystrophin isoform X1 [KO:K10366]
122107478  Des; desmin [KO:K07610]
122113601  Actb; actin, cytoplasmic 1 [KO:K05692]
122099409  Actg1; actin, cytoplasmic 2 [KO:K05692]
122114971  Ttn; titin [KO:K12567] [EC:2.7.11.1]
122126733  Tnnt2; troponin T, cardiac muscle isoform X1 [KO:K12045]
122107982  Tnnc1; troponin C, slow skeletal and cardiac muscles [KO:K05865]
122110728  Tnni3; troponin I, cardiac muscle [KO:K12044]
122114665  Actc1; actin, alpha cardiac muscle 1 [KO:K12314]
122114355  Tpm1; tropomyosin alpha-1 chain isoform X1 [KO:K10373]
122125925  tropomyosin alpha-1 chain-like [KO:K10373]
122122920  Tpm2; tropomyosin beta chain isoform X1 [KO:K10374]
122108101  tropomyosin alpha-3 chain-like [KO:K09290]
122109756  Tpm3; tropomyosin alpha-3 chain isoform X1 [KO:K09290]
122100886  Tpm4; tropomyosin alpha-4 chain isoform X1 [KO:K10375]
122105354  Mybpc3; myosin-binding protein C, cardiac-type [KO:K12568]
122095699  Myl3; myosin light chain 3 [KO:K12749]
122098548  Myl2; myosin regulatory light chain 2, ventricular/cardiac muscle isoform isoform X1 [KO:K10351]
122113382  myosin-6 [KO:K17751]
122113387  myosin-7 [KO:K17751]
122097016  Emd; emerin [KO:K12569]
122109350  Lmna; lamin [KO:K12641]
122115801  Adrb1; beta-1 adrenergic receptor [KO:K04141]
122124612  guanine nucleotide-binding protein G(s) subunit alpha isoform X1 [KO:K04632]
122124614  neuroendocrine secretory protein 55-like [KO:K04632]
122128067  Adcy1; adenylate cyclase type 1 [KO:K08041] [EC:4.6.1.1]
122120987  Adcy2; adenylate cyclase type 2 [KO:K08042] [EC:4.6.1.1]
122122500  Adcy3; adenylate cyclase type 3 isoform X1 [KO:K08043] [EC:4.6.1.1]
122113375  Adcy4; adenylate cyclase type 4 [KO:K08044] [EC:4.6.1.1]
122097950  Adcy5; adenylate cyclase type 5 [KO:K08045] [EC:4.6.1.1]
122106258  Adcy6; adenylate cyclase type 6 isoform X1 [KO:K08046] [EC:4.6.1.1]
122119361  Adcy7; adenylate cyclase type 7 isoform X1 [KO:K08047] [EC:4.6.1.1]
122125337  Adcy8; adenylate cyclase type 8 isoform X1 [KO:K08048] [EC:4.6.1.1]
122123783  Adcy9; adenylate cyclase type 9 isoform X1 [KO:K08049] [EC:4.6.1.1]
122104508  Prkacb; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
122119798  Prkaca; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
122108123  Cacna1d; voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851]
122098776  Cacna1f; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
122126705  Cacna1s; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
122103511  Cacnb1; voltage-dependent L-type calcium channel subunit beta-1 isoform X1 [KO:K04862]
122113099  Cacnb2; voltage-dependent L-type calcium channel subunit beta-2 isoform X1 [KO:K04863]
122106361  Cacnb3; voltage-dependent L-type calcium channel subunit beta-3 isoform X1 [KO:K04864]
122110846  Cacnb4; voltage-dependent L-type calcium channel subunit beta-4 isoform X1 [KO:K04865]
122111799  Cacna2d1; voltage-dependent calcium channel subunit alpha-2/delta-1 [KO:K04858]
122095552  Cacna2d2; voltage-dependent calcium channel subunit alpha-2/delta-2 [KO:K04859]
122108012  Cacna2d3; voltage-dependent calcium channel subunit alpha-2/delta-3 [KO:K04860]
122102546  voltage-dependent calcium channel subunit alpha-2/delta-4-like [KO:K04861]
122094975  Cacng1; voltage-dependent calcium channel gamma-1 subunit isoform X1 [KO:K04866]
122110569  voltage-dependent calcium channel gamma-2 subunit-like [KO:K04867]
122119286  Cacng2; voltage-dependent calcium channel gamma-2 subunit [KO:K04867]
122116183  Cacng3; voltage-dependent calcium channel gamma-3 subunit [KO:K04868]
122094950  Cacng4; voltage-dependent calcium channel gamma-4 subunit isoform X1 [KO:K04869]
122094956  Cacng5; voltage-dependent calcium channel gamma-5 subunit [KO:K04870]
122110699  Cacng6; voltage-dependent calcium channel gamma-6 subunit [KO:K04871]
122110697  Cacng7; voltage-dependent calcium channel gamma-7 subunit [KO:K04872]
122110693  Cacng8; voltage-dependent calcium channel gamma-8 subunit [KO:K04873]
122113232  Ryr2; ryanodine receptor 2 [KO:K04962]
122096216  Atp2a1; sarcoplasmic/endoplasmic reticulum calcium ATPase 1 isoform X1 [KO:K05853] [EC:7.2.2.10]
122098533  Atp2a2; sarcoplasmic/endoplasmic reticulum calcium ATPase 2 [KO:K05853] [EC:7.2.2.10]
122124839  Atp2a3; sarcoplasmic/endoplasmic reticulum calcium ATPase 3 isoform X1 [KO:K05853] [EC:7.2.2.10]
122107938  Pln; cardiac phospholamban [KO:K05852]
122118701  Slc8a3; sodium/calcium exchanger 3 [KO:K05849]
122104501  Slc8a1; sodium/calcium exchanger 1 isoform X1 [KO:K05849]
122125851  Slc8a2; sodium/calcium exchanger 2 isoform X1 [KO:K05849]
122119198  Igf1; insulin-like growth factor I isoform X1 [KO:K05459]
122097410  Tgfb1; transforming growth factor beta-1 proprotein [KO:K13375]
122109464  Tgfb2; transforming growth factor beta-2 proprotein isoform X1 [KO:K13376]
122118877  Tgfb3; transforming growth factor beta-3 proprotein isoform X1 [KO:K13377]
122111281  Tnf; tumor necrosis factor isoform X1 [KO:K03156]
122113292  Agt; angiotensinogen [KO:K09821]
Compound
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C01330  Sodium cation
Reference
PMID:12270949
  Authors
Fatkin D, Graham RM
  Title
Molecular mechanisms of inherited cardiomyopathies.
  Journal
Physiol Rev 82:945-80 (2002)
DOI:10.1152/physrev.00012.2002
Reference
PMID:18672201
  Authors
Lappe JM, Pelfrey CM, Tang WH
  Title
Recent insights into the role of autoimmunity in idiopathic dilated cardiomyopathy.
  Journal
J Card Fail 14:521-30 (2008)
DOI:10.1016/j.cardfail.2008.02.016
Reference
PMID:19273156
  Authors
Zhao P, Sharma AC, Ren J
  Title
Pathogenesis and therapy of autoimmunity-induced dilated cardiomyopathy.
  Journal
Front Biosci 14:1708-15 (2009)
DOI:10.2741/3334
Reference
PMID:11716909
  Authors
Franz WM, Muller OJ, Katus HA
  Title
Cardiomyopathies: from genetics to the prospect of treatment.
  Journal
Lancet 358:1627-37 (2001)
DOI:10.1016/S0140-6736(01)06657-0
Reference
PMID:11805847
  Authors
Towbin JA, Bowles NE
  Title
The failing heart.
  Journal
Nature 415:227-33 (2002)
DOI:10.1038/415227a
Reference
PMID:19017683
  Authors
Luk A, Ahn E, Soor GS, Butany J
  Title
Dilated cardiomyopathy: a review.
  Journal
J Clin Pathol 62:219-25 (2009)
DOI:10.1136/jcp.2008.060731
Reference
PMID:19246813
  Authors
Yoshikawa T, Baba A, Nagatomo Y
  Title
Autoimmune mechanisms underlying dilated cardiomyopathy.
  Journal
Circ J 73:602-7 (2009)
DOI:10.1253/circj.CJ-08-1151
Reference
PMID:14522402
  Authors
Mason JW
  Title
Myocarditis and dilated cardiomyopathy: an inflammatory link.
  Journal
Cardiovasc Res 60:5-10 (2003)
DOI:10.1016/S0008-6363(03)00437-1
Reference
PMID:15146239
  Authors
Jahns R, Boivin V, Hein L, Triebel S, Angermann CE, Ertl G, Lohse MJ
  Title
Direct evidence for a beta 1-adrenergic receptor-directed autoimmune attack as a cause of idiopathic dilated cardiomyopathy.
  Journal
J Clin Invest 113:1419-29 (2004)
DOI:10.1172/JCI20149
Reference
PMID:11524405
  Authors
Liu PP, Mason JW
  Title
Advances in the understanding of myocarditis.
  Journal
Circulation 104:1076-82 (2001)
DOI:10.1161/hc3401.095198
Reference
PMID:16817756
  Authors
Tam PE
  Title
Coxsackievirus myocarditis: interplay between virus and host in the pathogenesis of heart disease.
  Journal
Viral Immunol 19:133-46 (2006)
DOI:10.1089/vim.2006.19.133
Related
pathway
dsp04260  Cardiac muscle contraction
dsp04350  TGF-beta signaling pathway
dsp04512  ECM-receptor interaction
dsp04614  Renin-angiotensin system
dsp05416  Viral myocarditis
KO pathway
ko05414   
LinkDB

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