KEGG   PATHWAY: ocu05414
Entry
ocu05414                    Pathway                                
Name
Dilated cardiomyopathy - Oryctolagus cuniculus (rabbit)
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
ocu05414  Dilated cardiomyopathy
ocu05414

Disease
H00294  Dilated cardiomyopathy
Organism
Oryctolagus cuniculus (rabbit) [GN:ocu]
Gene
100008570  TTN; titin isoform X1 [KO:K12567] [EC:2.7.11.1]
100008642  [KO:K06485]
100008645  TGFB1; transforming growth factor beta-1 proprotein [KO:K13375]
100008668  IGF1; insulin-like growth factor I precursor [KO:K05459]
100008714  SSPN; sarcospan [KO:K22194]
100008734  ITGA2B; integrin alpha-IIb precursor [KO:K06476]
100008735  ITGB3; integrin beta-3 precursor [KO:K06493]
100008815  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
100008822  [KO:K05692]
100008898  [KO:K05719]
100008904  [KO:K04632]
100008956  [KO:K06487]
100009088  TNF; tumor necrosis factor precursor [KO:K03156]
100009097  [KO:K06856]
100009105  CACNA2D1; voltage-dependent calcium channel subunit alpha-2/delta-1 preproprotein [KO:K04858]
100009109  CACNB1; voltage-dependent L-type calcium channel subunit beta-1 [KO:K04862]
100009141  ITGB8; integrin beta-8 precursor [KO:K06591]
100009172  [KO:K06484]
100009178  SGCA; alpha-sarcoglycan precursor [KO:K12565]
100009179  SNTA1; alpha-1-syntrophin [KO:K24063]
100009201  [KO:K08042] [EC:4.6.1.1]
100009208  SGCB; beta-sarcoglycan [KO:K12566]
100009214  SGCG; gamma-sarcoglycan [KO:K12564]
100009243  NOS1; nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
100009263  CACNG1; voltage-dependent calcium channel gamma-1 subunit [KO:K04866]
100009272  ACTB; actin, cytoplasmic 1 [KO:K05692]
100009277  CACNB2; voltage-dependent L-type calcium channel subunit beta-2 [KO:K04863]
100009278  DAG1; dystroglycan 1 precursor [KO:K06265]
100009299  PLN; cardiac phospholamban [KO:K05852]
100009315  ADCY5; adenylate cyclase type 5 [KO:K08045] [EC:4.6.1.1]
100009371  TGFB2; transforming growth factor beta-2 proprotein precursor [KO:K13376]
100009402  CACNB3; voltage-dependent L-type calcium channel subunit beta-3 [KO:K04864]
100009536  RYR2; ryanodine receptor 2 [KO:K04962]
100009585  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
100037716  ATP2A1; sarcoplasmic/endoplasmic reticulum calcium ATPase 1 [KO:K05853] [EC:7.2.2.10]
100038308  ATP2A2; sarcoplasmic/endoplasmic reticulum calcium ATPase 2 [KO:K05853] [EC:7.2.2.10]
100101555  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
100101566  MYH7; myosin-7 [KO:K17751]
100101587  [KO:K06525]
100101621  [KO:K06481]
100125984  TPM2; tropomyosin beta chain [KO:K10374]
100125987  MYH6; myosin-6 [KO:K17751]
100125989  TPM1; tropomyosin alpha-1 chain [KO:K10373]
100327259  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
100328570  SLC8A1; sodium/calcium exchanger 1 precursor [KO:K05849]
100328755  EMD; emerin [KO:K12569]
100328774  CACNG7; voltage-dependent calcium channel gamma-7 subunit [KO:K04872]
100328890  DES; desmin [KO:K07610]
100337882  [KO:K08048] [EC:4.6.1.1]
100338084  [KO:K04141]
100338232  [KO:K06589]
100338772  [KO:K04869]
100339021  [KO:K04870]
100339605  [KO:K05849]
100339625  [KO:K06585]
100340248  [KO:K06586]
100340339  [KO:K12314]
100340494  [KO:K04859]
100340877  [KO:K08049] [EC:4.6.1.1]
100341037  [KO:K05692]
100342212  [KO:K04345] [EC:2.7.11.11]
100342711  [KO:K06480]
100342999  [KO:K12959]
100343175  [KO:K10375]
100343574  [KO:K12641]
100343958  [KO:K08043] [EC:4.6.1.1]
100344224  [KO:K04865]
100344251  [KO:K06587]
100346005  [KO:K06583]
100346103  [KO:K06483]
100346323  [KO:K04632]
100346742  [KO:K08044] [EC:4.6.1.1]
100346773  [KO:K12568]
100347570  [KO:K05865]
100347740  [KO:K12749]
100348594  [KO:K06482]
100349537  [KO:K27062]
100349893  [KO:K09290]
100350075  [KO:K04868]
100350483  [KO:K04867]
100350500  [KO:K05637]
100350557  [KO:K06590]
100351181  [KO:K06584]
100351233  [KO:K12563]
100351327  [KO:K04860]
100351412  [KO:K26998]
100351420  [KO:K09290]
100351877  [KO:K04871]
100352301  [KO:K13375]
100352334  [KO:K27061]
100352996  [KO:K06856]
100353304  [KO:K10351]
100353328  [KO:K06856]
100353546  [KO:K06588]
100354108  [KO:K04861]
100354514  [KO:K24064]
100354934  [KO:K08046] [EC:4.6.1.1]
100354976  [KO:K05853] [EC:7.2.2.10]
100355731  [KO:K10366]
100357261  [KO:K04853]
100357357  [KO:K08047] [EC:4.6.1.1]
100357482  [KO:K05637]
100358371  [KO:K09821]
100358483  [KO:K05849]
100358982  [KO:K13377]
103345448  [KO:K13375]
103346112  [KO:K04873]
103347035  [KO:K08041] [EC:4.6.1.1]
103347220  [KO:K06856]
103348511  [KO:K24064]
103350383  [KO:K06585]
127487418  [KO:K06583]
Compound
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C01330  Sodium cation
Reference
  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
  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
  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
  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
  Authors
Towbin JA, Bowles NE
  Title
The failing heart.
  Journal
Nature 415:227-33 (2002)
DOI:10.1038/415227a
Reference
  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
  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
  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
  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
  Authors
Liu PP, Mason JW
  Title
Advances in the understanding of myocarditis.
  Journal
Circulation 104:1076-82 (2001)
DOI:10.1161/hc3401.095198
Reference
  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
ocu04260  Cardiac muscle contraction
ocu04350  TGF-beta signaling pathway
ocu04512  ECM-receptor interaction
ocu04614  Renin-angiotensin system
ocu05416  Viral myocarditis
KO pathway
ko05414   

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