KEGG   PATHWAY: rbb05414
Entry
rbb05414                    Pathway                                
Name
Dilated cardiomyopathy - Rhinopithecus bieti (black snub-nosed monkey)
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
rbb05414  Dilated cardiomyopathy
rbb05414

Organism
Rhinopithecus bieti (black snub-nosed monkey) [GN:rbb]
Gene
108522901  ITGA1; integrin alpha-1 [KO:K06480]
108522903  ITGA2; integrin alpha-2 [KO:K06481]
108532191  ITGA2B; integrin alpha-IIb isoform X1 [KO:K06476]
108516444  ITGA3; integrin alpha-3 isoform X1 [KO:K06482]
108526617  integrin alpha-4 [KO:K06483]
108514611  ITGA5; integrin alpha-5 [KO:K06484]
108522273  ITGA6; integrin alpha-6 isoform X1 [KO:K06485]
108516025  ITGA7; integrin alpha-7 isoform X1 [KO:K06583]
108530933  ITGA8; integrin alpha-8 isoform X1 [KO:K06584]
108524661  ITGA9; LOW QUALITY PROTEIN: integrin alpha-9 [KO:K06585]
108531442  ITGA10; integrin alpha-10 isoform X1 [KO:K06586]
108514729  ITGA11; integrin alpha-11 isoform X1 [KO:K06587]
108535417  ITGAV; integrin alpha-V isoform X1 [KO:K06487]
108529400  ITGB1; integrin beta-1 [KO:K05719]
108536294  ITGB3; integrin beta-3 isoform X1 [KO:K06493]
108537357  ITGB4; integrin beta-4 isoform X1 [KO:K06525]
108517420  ITGB5; integrin beta-5 [KO:K06588]
108527702  ITGB6; LOW QUALITY PROTEIN: integrin beta-6 [KO:K06589]
108521588  ITGB7; integrin beta-7 [KO:K06590]
108529317  ITGB8; LOW QUALITY PROTEIN: integrin beta-8 [KO:K06591]
108523734  SSPN; sarcospan isoform X1 [KO:K22194]
108516439  SGCA; alpha-sarcoglycan isoform X1 [KO:K12565]
108533538  SGCB; beta-sarcoglycan [KO:K12566]
108531173  SGCG; LOW QUALITY PROTEIN: gamma-sarcoglycan [KO:K12564]
108516771  SGCD; LOW QUALITY PROTEIN: delta-sarcoglycan [KO:K12563]
108517184  SGCE; epsilon-sarcoglycan isoform X1 [KO:K27061]
108533115  SGCZ; zeta-sarcoglycan [KO:K27062]
108534366  LAMA2; laminin subunit alpha-2 [KO:K05637]
108523928  LAMA1; laminin subunit alpha-1 isoform X1 [KO:K05637]
108526986  DAG1; LOW QUALITY PROTEIN: dystroglycan [KO:K06265]
108530950  CAV3; caveolin-3 [KO:K12959]
108539288  NOS1; LOW QUALITY PROTEIN: nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
108536985  SNTA1; alpha-1-syntrophin [KO:K24063]
108512298  SNTB1; beta-1-syntrophin [KO:K24064]
108530907  DTNA; dystrobrevin alpha isoform X1 [KO:K26998]
108518459  DES; desmin [KO:K07610]
108534125  ACTG1; actin, cytoplasmic 2 [KO:K05692]
108538237  ACTB; actin, cytoplasmic 1 [KO:K05692]
108527098  TTN; LOW QUALITY PROTEIN: titin [KO:K12567] [EC:2.7.11.1]
108545081  TNNT2; troponin T, cardiac muscle isoform X1 [KO:K12045]
108522336  TNNC1; troponin C, slow skeletal and cardiac muscles isoform X1 [KO:K05865]
108542529  TNNI3; troponin I, cardiac muscle isoform X1 [KO:K12044]
108543689  ACTC1; actin, alpha cardiac muscle 1 [KO:K12314]
108527657  TPM1; tropomyosin alpha-1 chain isoform X1 [KO:K10373]
108518998  TPM2; tropomyosin beta chain isoform X1 [KO:K10374]
108517668  TPM3; tropomyosin alpha-3 chain isoform X1 [KO:K09290]
108517077  tropomyosin alpha-3 chain-like [KO:K09290]
108543293  TPM4; tropomyosin alpha-4 chain isoform X1 [KO:K10375]
108521947  MYBPC3; myosin-binding protein C, cardiac-type [KO:K12568]
108517652  MYL3; myosin light chain 3 [KO:K12749]
108512247  MYL2; myosin regulatory light chain 2, ventricular/cardiac muscle isoform [KO:K10351]
108523551  myosin-7 [KO:K17751]
108523597  MYH6; LOW QUALITY PROTEIN: myosin-6 [KO:K17751]
108518767  EMD; emerin [KO:K12569]
108515510  LMNA; lamin isoform X1 [KO:K12641]
108522153  putative V-set and immunoglobulin domain-containing-like protein IGHV4OR15-8 [KO:K06856]
108511997  putative V-set and immunoglobulin domain-containing-like protein IGHV4OR15-8 [KO:K06856]
108512001  putative V-set and immunoglobulin domain-containing-like protein IGHV4OR15-8 isoform X1 [KO:K06856]
108520828  uncharacterized protein LOC108520828 [KO:K06856]
108519975  uncharacterized protein LOC108519975 [KO:K06856]
108519976  LOW QUALITY PROTEIN: uncharacterized protein LOC108519976 [KO:K06856]
108519983  uncharacterized protein LOC108519983 [KO:K06856]
108519992  putative V-set and immunoglobulin domain-containing-like protein IGHV4OR15-8 [KO:K06856]
108524775  putative V-set and immunoglobulin domain-containing-like protein IGHV4OR15-8 [KO:K06856]
108542470  ADRB1; LOW QUALITY PROTEIN: beta-1 adrenergic receptor [KO:K04141]
108541624  GNAS; guanine nucleotide-binding protein G(s) subunit alpha isoforms XLas isoform X1 [KO:K04632]
108541626  neuroendocrine secretory protein 55 [KO:K04632]
108534793  ADCY1; adenylate cyclase type 1 isoform X1 [KO:K08041] [EC:4.6.1.1]
108536960  ADCY2; adenylate cyclase type 2 isoform X1 [KO:K08042] [EC:4.6.1.1]
108543021  ADCY3; adenylate cyclase type 3 isoform X1 [KO:K08043] [EC:4.6.1.1]
108540946  ADCY4; adenylate cyclase type 4 isoform X1 [KO:K08044] [EC:4.6.1.1]
108517430  ADCY5; LOW QUALITY PROTEIN: adenylate cyclase type 5 [KO:K08045] [EC:4.6.1.1]
108543933  ADCY6; adenylate cyclase type 6 [KO:K08046] [EC:4.6.1.1]
108522948  ADCY7; adenylate cyclase type 7 isoform X1 [KO:K08047] [EC:4.6.1.1]
108514094  ADCY8; adenylate cyclase type 8 [KO:K08048] [EC:4.6.1.1]
108538326  ADCY9; adenylate cyclase type 9 isoform X1 [KO:K08049] [EC:4.6.1.1]
108514761  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
108540003  cAMP-dependent protein kinase catalytic subunit alpha-like [KO:K04345] [EC:2.7.11.11]
108527683  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
108530264  PRKACG; cAMP-dependent protein kinase catalytic subunit gamma [KO:K04345] [EC:2.7.11.11]
108522695  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
108515198  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
108521361  voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
108545075  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857]
108533973  CACNB1; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit beta-1 [KO:K04862]
108527755  CACNB2; voltage-dependent L-type calcium channel subunit beta-2 isoform X1 [KO:K04863]
108543940  CACNB3; voltage-dependent L-type calcium channel subunit beta-3 isoform X1 [KO:K04864]
108525018  CACNB4; voltage-dependent L-type calcium channel subunit beta-4 isoform X1 [KO:K04865]
108536908  CACNA2D1; voltage-dependent calcium channel subunit alpha-2/delta-1 isoform X1 [KO:K04858]
108526929  CACNA2D2; voltage-dependent calcium channel subunit alpha-2/delta-2 [KO:K04859]
108515798  CACNA2D3; voltage-dependent calcium channel subunit alpha-2/delta-3 isoform X1 [KO:K04860]
108522308  CACNA2D4; voltage-dependent calcium channel subunit alpha-2/delta-4 [KO:K04861]
108517742  CACNG1; voltage-dependent calcium channel gamma-1 subunit [KO:K04866]
108518485  CACNG2; voltage-dependent calcium channel gamma-2 subunit [KO:K04867]
108538878  CACNG3; voltage-dependent calcium channel gamma-3 subunit [KO:K04868]
108517736  CACNG4; voltage-dependent calcium channel gamma-4 subunit [KO:K04869]
108517743  CACNG5; voltage-dependent calcium channel gamma-5 subunit [KO:K04870]
108529454  CACNG6; voltage-dependent calcium channel gamma-6 subunit isoform X1 [KO:K04871]
108529453  CACNG7; voltage-dependent calcium channel gamma-7 subunit isoform X1 [KO:K04872]
108529445  CACNG8; voltage-dependent calcium channel gamma-8 subunit [KO:K04873]
108522194  RYR2; LOW QUALITY PROTEIN: ryanodine receptor 2 [KO:K04962]
108523688  ATP2A1; sarcoplasmic/endoplasmic reticulum calcium ATPase 1 [KO:K05853] [EC:7.2.2.10]
108520719  ATP2A3; sarcoplasmic/endoplasmic reticulum calcium ATPase 3 isoform X1 [KO:K05853] [EC:7.2.2.10]
108534362  ATP2A2; sarcoplasmic/endoplasmic reticulum calcium ATPase 2 [KO:K05853] [EC:7.2.2.10]
108544714  PLN; cardiac phospholamban [KO:K05852]
108542784  SLC8A1; sodium/calcium exchanger 1 isoform X1 [KO:K05849]
108520278  SLC8A3; sodium/calcium exchanger 3 isoform X1 [KO:K05849]
108540286  SLC8A2; LOW QUALITY PROTEIN: sodium/calcium exchanger 2 [KO:K05849]
108543134  IGF1; insulin-like growth factor I isoform X1 [KO:K05459]
108536158  TGFB1; transforming growth factor beta-1 isoform X1 [KO:K13375]
108528282  TGFB2; transforming growth factor beta-2 isoform X1 [KO:K13376]
108534939  TGFB3; transforming growth factor beta-3 [KO:K13377]
108520255  TNF; tumor necrosis factor isoform X1 [KO:K03156]
108537839  AGT; LOW QUALITY PROTEIN: 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
rbb04260  Cardiac muscle contraction
rbb04350  TGF-beta signaling pathway
rbb04512  ECM-receptor interaction
rbb04614  Renin-angiotensin system
rbb05416  Viral myocarditis
KO pathway
ko05414   
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