KEGG   PATHWAY: ray05414
ray05414                    Pathway                                

Dilated cardiomyopathy - Rousettus aegyptiacus (Egyptian rousette)
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.
Human Diseases; Cardiovascular disease
Pathway map
ray05414  Dilated cardiomyopathy

Rousettus aegyptiacus (Egyptian rousette) [GN:ray]
107509664  ITGA1; LOW QUALITY PROTEIN: integrin alpha-1 [KO:K06480]
107509660  ITGA2; integrin alpha-2 [KO:K06481]
107508144  ITGA2B; integrin alpha-IIb isoform X1 [KO:K06476]
107517588  ITGA3; integrin alpha-3 [KO:K06482]
107520625  ITGA4; integrin alpha-4 [KO:K06483]
107521082  ITGA5; integrin alpha-5 isoform X1 [KO:K06484]
107505202  integrin alpha-6 isoform X1 [KO:K06485]
107521260  ITGA7; integrin alpha-7 isoform X1 [KO:K06583]
107504826  ITGA8; integrin alpha-8 [KO:K06584]
107499593  ITGA9; integrin alpha-9 [KO:K06585]
107510088  ITGA10; LOW QUALITY PROTEIN: integrin alpha-10 [KO:K06586]
107508924  ITGA11; integrin alpha-11 [KO:K06587]
107513835  ITGAV; integrin alpha-V isoform X1 [KO:K06487]
107499295  ITGB1; integrin beta-1 isoform X1 [KO:K05719]
107501647  integrin beta-3 [KO:K06493]
107501601  ITGB4; integrin beta-4 [KO:K06525]
107505238  ITGB5; integrin beta-5 [KO:K06588]
107514780  ITGB6; integrin beta-6 isoform X1 [KO:K06589]
107509843  ITGB7; integrin beta-7 isoform X1 [KO:K06590]
107518140  ITGB8; integrin beta-8 isoform X1 [KO:K06591]
107521917  SGCD; delta-sarcoglycan [KO:K12563]
107507608  gamma-sarcoglycan-like isoform X1 [KO:K12564]
107519710  SGCG; gamma-sarcoglycan [KO:K12564]
107517617  SGCA; alpha-sarcoglycan [KO:K12565]
107513944  SGCB; LOW QUALITY PROTEIN: beta-sarcoglycan [KO:K12566]
107497012  LAMA2; laminin subunit alpha-2 [KO:K05637]
107498184  LAMA1; LOW QUALITY PROTEIN: laminin subunit alpha-1 [KO:K05637]
107513352  DAG1; dystroglycan [KO:K06265]
107516772  DES; desmin [KO:K07610]
107520262  ACTG1; actin, cytoplasmic 2 [KO:K05692]
107515934  ACTB; actin, cytoplasmic 1 [KO:K05692]
107514656  TTN; titin isoform X1 [KO:K12567] [EC:]
107513651  TNNT2; troponin T, cardiac muscle isoform X1 [KO:K12045]
107498574  TNNC1; troponin C, slow skeletal and cardiac muscles [KO:K05865]
107515439  TNNI3; troponin I, cardiac muscle [KO:K12044]
107508080  ACTC1; actin, alpha cardiac muscle 1 [KO:K12314]
107508997  TPM1; tropomyosin alpha-1 chain isoform X1 [KO:K10373]
107502868  TPM2; tropomyosin beta chain isoform X1 [KO:K10374]
107509189  TPM3; tropomyosin alpha-3 chain isoform X1 [KO:K09290]
107517241  TPM4; tropomyosin alpha-4 chain isoform X1 [KO:K10375]
107511848  MYBPC3; myosin-binding protein C, cardiac-type isoform X1 [KO:K12568]
107509109  MYL3; myosin light chain 3 [KO:K12749]
107513248  MYL2; myosin regulatory light chain 2, ventricular/cardiac muscle isoform [KO:K10351]
107498010  MYH6; myosin-6 [KO:K17751]
107498011  myosin-7 [KO:K17751]
107521499  LMNA; lamin isoform X1 [KO:K12641]
107507577  Ig heavy chain Mem5-like [KO:K06856]
107511714  ADRB1; beta-1 adrenergic receptor [KO:K04141]
107500068  neuroendocrine secretory protein 55-like [KO:K04632]
107500067  GNAS; guanine nucleotide-binding protein G(s) subunit alpha isoform X1 [KO:K04632]
107512406  ADCY1; adenylate cyclase type 1 [KO:K08041] [EC:]
107502700  ADCY2; LOW QUALITY PROTEIN: adenylate cyclase type 2 [KO:K08042] [EC:]
107498506  ADCY3; LOW QUALITY PROTEIN: adenylate cyclase type 3 [KO:K08043] [EC:]
107498014  ADCY4; LOW QUALITY PROTEIN: adenylate cyclase type 4 [KO:K08044] [EC:]
107518038  ADCY5; LOW QUALITY PROTEIN: adenylate cyclase type 5 [KO:K08045] [EC:]
107514224  ADCY6; adenylate cyclase type 6 isoform X1 [KO:K08046] [EC:]
107512248  ADCY7; adenylate cyclase type 7 isoform X1 [KO:K08047] [EC:]
107497689  ADCY8; adenylate cyclase type 8 isoform X1 [KO:K08048] [EC:]
107521680  ADCY9; adenylate cyclase type 9 [KO:K08049] [EC:]
107519437  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:]
107511590  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:]
107500540  CACNA1C; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
107516535  CACNA1D; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851]
107514999  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
107513628  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
107520701  CACNB1; voltage-dependent L-type calcium channel subunit beta-1 isoform X1 [KO:K04862]
107513663  CACNB2; voltage-dependent L-type calcium channel subunit beta-2 isoform X1 [KO:K04863]
107514227  CACNB3; voltage-dependent L-type calcium channel subunit beta-3 isoform X1 [KO:K04864]
107513916  CACNB4; voltage-dependent L-type calcium channel subunit beta-4 isoform X1 [KO:K04865]
107501281  CACNA2D1; voltage-dependent calcium channel subunit alpha-2/delta-1 isoform X1 [KO:K04858]
107513293  CACNA2D2; LOW QUALITY PROTEIN: voltage-dependent calcium channel subunit alpha-2/delta-2 [KO:K04859]
107516533  CACNA2D3; LOW QUALITY PROTEIN: voltage-dependent calcium channel subunit alpha-2/delta-3 [KO:K04860]
107500543  CACNA2D4; voltage-dependent calcium channel subunit alpha-2/delta-4 [KO:K04861]
107510248  CACNG1; voltage-dependent calcium channel gamma-1 subunit [KO:K04866]
107519239  CACNG2; voltage-dependent calcium channel gamma-2 subunit [KO:K04867]
107508836  CACNG3; voltage-dependent calcium channel gamma-3 subunit [KO:K04868]
107510310  CACNG4; voltage-dependent calcium channel gamma-4 subunit [KO:K04869]
107510308  CACNG5; voltage-dependent calcium channel gamma-5 subunit [KO:K04870]
107503034  CACNG6; voltage-dependent calcium channel gamma-6 subunit [KO:K04871]
107503063  CACNG7; voltage-dependent calcium channel gamma-7 subunit [KO:K04872]
107503036  CACNG8; LOW QUALITY PROTEIN: voltage-dependent calcium channel gamma-8 subunit [KO:K04873]
107520024  RYR2; LOW QUALITY PROTEIN: ryanodine receptor 2 [KO:K04962]
107515804  ATP2A1; sarcoplasmic/endoplasmic reticulum calcium ATPase 1 isoform X1 [KO:K05853] [EC:]
107507669  ATP2A3; sarcoplasmic/endoplasmic reticulum calcium ATPase 3 isoform X1 [KO:K05853] [EC:]
107513235  ATP2A2; sarcoplasmic/endoplasmic reticulum calcium ATPase 2 [KO:K05853] [EC:]
107515126  PLN; cardiac phospholamban [KO:K05852]
107503262  SLC8A3; sodium/calcium exchanger 3 isoform X1 [KO:K05849]
107516018  SLC8A2; sodium/calcium exchanger 2 isoform X1 [KO:K05849]
107509443  SLC8A1; sodium/calcium exchanger 1 isoform X1 [KO:K05849]
107501166  IGF1; insulin-like growth factor I isoform X1 [KO:K05459]
107505490  TGFB1; transforming growth factor beta-1 [KO:K13375]
107520844  TGFB2; transforming growth factor beta-2 isoform X1 [KO:K13376]
107498899  TGFB3; transforming growth factor beta-3 [KO:K13377]
107498497  TNF; tumor necrosis factor [KO:K03156]
107520073  AGT; LOW QUALITY PROTEIN: angiotensinogen [KO:K09821]
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C01330  Sodium cation
Fatkin D, Graham RM
Molecular mechanisms of inherited cardiomyopathies.
Physiol Rev 82:945-80 (2002)
Lappe JM, Pelfrey CM, Tang WH
Recent insights into the role of autoimmunity in idiopathic dilated cardiomyopathy.
J Card Fail 14:521-30 (2008)
Zhao P, Sharma AC, Ren J
Pathogenesis and therapy of autoimmunity-induced dilated cardiomyopathy.
Front Biosci 14:1708-15 (2009)
Franz WM, Muller OJ, Katus HA
Cardiomyopathies: from genetics to the prospect of treatment.
Lancet 358:1627-37 (2001)
Towbin JA, Bowles NE
The failing heart.
Nature 415:227-33 (2002)
Luk A, Ahn E, Soor GS, Butany J
Dilated cardiomyopathy: a review.
J Clin Pathol 62:219-25 (2009)
Yoshikawa T, Baba A, Nagatomo Y
Autoimmune mechanisms underlying dilated cardiomyopathy.
Circ J 73:602-7 (2009)
Mason JW
Myocarditis and dilated cardiomyopathy: an inflammatory link.
Cardiovasc Res 60:5-10 (2003)
Jahns R, Boivin V, Hein L, Triebel S, Angermann CE, Ertl G, Lohse MJ
Direct evidence for a beta 1-adrenergic receptor-directed autoimmune attack as a cause of idiopathic dilated cardiomyopathy.
J Clin Invest 113:1419-29 (2004)
Liu PP, Mason JW
Advances in the understanding of myocarditis.
Circulation 104:1076-82 (2001)
Tam PE
Coxsackievirus myocarditis: interplay between virus and host in the pathogenesis of heart disease.
Viral Immunol 19:133-46 (2006)
ray04260  Cardiac muscle contraction
ray04350  TGF-beta signaling pathway
ray04512  ECM-receptor interaction
ray04614  Renin-angiotensin system
ray05416  Viral myocarditis
KO pathway

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