KEGG   PATHWAY: mpuf05414
Entry
mpuf05414                   Pathway                                
Name
Dilated cardiomyopathy - Mustela putorius furo (domestic ferret)
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
mpuf05414  Dilated cardiomyopathy
mpuf05414

Organism
Mustela putorius furo (domestic ferret) [GN:mpuf]
Gene
101677795  ITGA1; integrin alpha-1 isoform X1 [KO:K06480]
101676920  ITGA2; integrin alpha-2 isoform X1 [KO:K06481]
101678224  ITGA2B; integrin alpha-IIb isoform X2 [KO:K06476]
101684180  ITGA3; integrin alpha-3 isoform X1 [KO:K06482]
101681396  ITGA4; integrin alpha-4 [KO:K06483]
101686447  ITGA5; integrin alpha-5 [KO:K06484]
101682530  ITGA6; integrin alpha-6 isoform X1 [KO:K06485]
101692696  ITGA7; integrin alpha-7 isoform X2 [KO:K06583]
101692829  ITGA8; integrin alpha-8 [KO:K06584]
101669936  ITGA9; integrin alpha-9 [KO:K06585]
101686096  ITGA10; integrin alpha-10 isoform X4 [KO:K06586]
101672159  ITGA11; integrin alpha-11 [KO:K06587]
101681626  ITGAV; integrin alpha-V [KO:K06487]
101690880  LOW QUALITY PROTEIN: integrin alpha-V-like [KO:K06487]
101685180  ITGB1; integrin beta-1 isoform X1 [KO:K05719]
101683751  ITGB3; integrin beta-3 [KO:K06493]
101675802  ITGB4; integrin beta-4 isoform X1 [KO:K06525]
101671893  ITGB5; integrin beta-5 [KO:K06588]
101690894  ITGB6; integrin beta-6 isoform X1 [KO:K06589]
101685685  ITGB7; integrin beta-7 [KO:K06590]
101675667  ITGB8; integrin beta-8 isoform X1 [KO:K06591]
101683259  SSPN; sarcospan isoform X1 [KO:K22194]
101686508  SGCA; alpha-sarcoglycan isoform X1 [KO:K12565]
101676646  SGCB; beta-sarcoglycan [KO:K12566]
101676694  SGCG; gamma-sarcoglycan isoform X1 [KO:K12564]
101684263  SGCD; delta-sarcoglycan isoform X1 [KO:K12563]
101689363  SGCE; epsilon-sarcoglycan isoform X1 [KO:K27061]
101671937  SGCZ; zeta-sarcoglycan isoform X1 [KO:K27062]
101673993  LAMA1; laminin subunit alpha-1 [KO:K05637]
101687362  LAMA2; laminin subunit alpha-2 [KO:K05637]
101677695  DAG1; dystroglycan [KO:K06265]
101684840  CAV3; caveolin-3 [KO:K12959]
101692439  NOS1; nitric oxide synthase, brain isoform X1 [KO:K13240] [EC:1.14.13.39]
101685154  SNTA1; alpha-1-syntrophin [KO:K24063]
106003342  SNTB1; beta-1-syntrophin [KO:K24064]
101676330  beta-1-syntrophin [KO:K24064]
101676859  beta-2-syntrophin [KO:K24064]
101674122  DTNA; dystrobrevin alpha isoform X1 [KO:K26998]
101672364  DMD; dystrophin [KO:K10366]
101682328  DES; desmin [KO:K07610]
101679540  ACTG1; actin, cytoplasmic 2 [KO:K05692]
101686268  ACTB; actin, cytoplasmic 1 [KO:K05692]
101687481  TTN; titin [KO:K12567] [EC:2.7.11.1]
101690457  TNNT2; troponin T, cardiac muscle isoform X1 [KO:K12045]
101680196  TNNC1; troponin C, slow skeletal and cardiac muscles [KO:K05865]
106003394  TNNI3; troponin I, cardiac muscle [KO:K12044]
101684977  ACTC1; actin, alpha cardiac muscle 1 [KO:K12314]
101686383  TPM1; tropomyosin alpha-1 chain isoform X1 [KO:K10373]
101678214  TPM2; tropomyosin beta chain isoform X1 [KO:K10374]
101681825  TPM3; tropomyosin alpha-3 chain [KO:K09290]
101688400  TPM4; tropomyosin alpha-4 chain isoform X1 [KO:K10375]
101690976  MYBPC3; myosin-binding protein C, cardiac-type [KO:K12568]
101686212  MYL3; myosin light chain 3 [KO:K12749]
101670913  MYL2; myosin regulatory light chain 2, ventricular/cardiac muscle isoform [KO:K10351]
101673577  myosin-7 [KO:K17751]
101673860  myosin-6 [KO:K17751]
101689231  EMD; emerin [KO:K12569]
101685893  LMNA; lamin [KO:K12641]
101694142  guanine nucleotide-binding protein G(s) subunit alpha [KO:K04632]
106003427  neuroendocrine secretory protein 55 [KO:K04632]
101676834  ADCY1; adenylate cyclase type 1 isoform X1 [KO:K08041] [EC:4.6.1.1]
101674868  ADCY2; LOW QUALITY PROTEIN: adenylate cyclase type 2 [KO:K08042] [EC:4.6.1.1]
101671296  ADCY3; adenylate cyclase type 3 isoform X1 [KO:K08043] [EC:4.6.1.1]
101676211  ADCY4; adenylate cyclase type 4 isoform X1 [KO:K08044] [EC:4.6.1.1]
101673819  ADCY5; adenylate cyclase type 5 [KO:K08045] [EC:4.6.1.1]
101676713  ADCY6; adenylate cyclase type 6 [KO:K08046] [EC:4.6.1.1]
101684190  ADCY7; adenylate cyclase type 7 isoform X1 [KO:K08047] [EC:4.6.1.1]
101693988  ADCY8; adenylate cyclase type 8 isoform X1 [KO:K08048] [EC:4.6.1.1]
101680942  ADCY9; adenylate cyclase type 9 [KO:K08049] [EC:4.6.1.1]
101692436  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
101681986  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
101679746  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X10 [KO:K04850]
101669870  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
101689134  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
101688476  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857]
101677145  CACNB1; voltage-dependent L-type calcium channel subunit beta-1 isoform X1 [KO:K04862]
101674245  CACNB2; voltage-dependent L-type calcium channel subunit beta-2 isoform X1 [KO:K04863]
101675014  CACNB3; voltage-dependent L-type calcium channel subunit beta-3 isoform X1 [KO:K04864]
101673997  CACNB4; voltage-dependent L-type calcium channel subunit beta-4 isoform X1 [KO:K04865]
101678500  CACNA2D1; voltage-dependent calcium channel subunit alpha-2/delta-1 isoform X1 [KO:K04858]
101681641  CACNA2D2; voltage-dependent calcium channel subunit alpha-2/delta-2 isoform X1 [KO:K04859]
101675438  CACNA2D3; voltage-dependent calcium channel subunit alpha-2/delta-3 [KO:K04860]
101678949  CACNA2D4; voltage-dependent calcium channel subunit alpha-2/delta-4 [KO:K04861]
101681317  CACNG1; voltage-dependent calcium channel gamma-1 subunit [KO:K04866]
101693507  CACNG2; voltage-dependent calcium channel gamma-2 subunit [KO:K04867]
101675211  CACNG3; voltage-dependent calcium channel gamma-3 subunit [KO:K04868]
101681593  CACNG4; voltage-dependent calcium channel gamma-4 subunit [KO:K04869]
101680034  CACNG5; voltage-dependent calcium channel gamma-5 subunit [KO:K04870]
101689811  CACNG6; voltage-dependent calcium channel gamma-6 subunit [KO:K04871]
101672035  CACNG7; voltage-dependent calcium channel gamma-7 subunit [KO:K04872]
101689510  CACNG8; voltage-dependent calcium channel gamma-8 subunit [KO:K04873]
101677570  RYR2; ryanodine receptor 2 isoform X1 [KO:K04962]
101674156  ATP2A3; sarcoplasmic/endoplasmic reticulum calcium ATPase 3 isoform X1 [KO:K05853] [EC:7.2.2.10]
101688541  ATP2A1; sarcoplasmic/endoplasmic reticulum calcium ATPase 1 isoform X1 [KO:K05853] [EC:7.2.2.10]
101690442  ATP2A2; sarcoplasmic/endoplasmic reticulum calcium ATPase 2 [KO:K05853] [EC:7.2.2.10]
101682416  PLN; cardiac phospholamban [KO:K05852]
101680809  SLC8A3; sodium/calcium exchanger 3 isoform X1 [KO:K05849]
101693907  SLC8A1; sodium/calcium exchanger 1 isoform X1 [KO:K05849]
101687861  SLC8A2; sodium/calcium exchanger 2 [KO:K05849]
101680922  IGF1; insulin-like growth factor I isoform X1 [KO:K05459]
101670127  TGFB1; transforming growth factor beta-1 isoform X1 [KO:K13375]
101677182  TGFB2; transforming growth factor beta-2 isoform X1 [KO:K13376]
101678400  TGFB3; transforming growth factor beta-3 [KO:K13377]
101687148  TNF; tumor necrosis factor isoform X1 [KO:K03156]
101675397  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
mpuf04260  Cardiac muscle contraction
mpuf04350  TGF-beta signaling pathway
mpuf04512  ECM-receptor interaction
mpuf04614  Renin-angiotensin system
mpuf05416  Viral myocarditis
KO pathway
ko05414   
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