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Entry
ko05414                     Pathway                                

Name
Dilated cardiomyopathy
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 diseases
BRITE hierarchy
Pathway map
Dilated cardiomyopathy
ko05414

All organismsOrtholog table
Disease
H00294  
Dilated cardiomyopathy (DCM)
H00728  
Brugada syndrome (BRS)
Orthology
K06480  
integrin alpha 1
K06481  
integrin alpha 2
K06476  
integrin alpha 2B
K06482  
integrin alpha 3
K06483  
integrin alpha 4
K06484  
integrin alpha 5
K06485  
integrin alpha 6
K06583  
integrin alpha 7
K06584  
integrin alpha 8
K06585  
integrin alpha 9
K06586  
integrin alpha 10
K06587  
integrin alpha 11
K06487  
integrin alpha V
K05719  
integrin beta 1
K06493  
integrin beta 3
K06525  
integrin beta 4
K06588  
integrin beta 5
K06589  
integrin beta 6
K06590  
integrin beta 7
K06591  
integrin beta 8
K12563  
delta-sarcoglycan
K12564  
gamma-sarcoglycan
K12565  
alpha-sarcoglycan
K12566  
beta-sarcoglycan
K06265  
dystroglycan 1
K07610  
desmin
K10366  
dystrophin
K05692  
actin beta/gamma 1
K12567  
titin [EC:2.7.11.1]
K12045  
troponin T, cardiac muscle
K05865  
troponin C, slow skeletal and cardiac muscles
K12044  
troponin I, cardiac muscle
K12314  
actin, alpha cardiac muscle
K10373  
tropomyosin 1
K10374  
tropomyosin 2
K09290  
tropomyosin 3
K10375  
tropomyosin 4
K12568  
myosin-binding protein C, cardiac-type
K12749  
myosin light chain 3
K10351  
myosin regulatory light chain 2
K12569  
emerin
K12641  
lamin A/C
K06856  
immunoglobulin heavy chain
K04141  
adrenergic receptor beta-1
K04632  
guanine nucleotide-binding protein G(s) subunit alpha
K08041  
adenylate cyclase 1 [EC:4.6.1.1]
K08042  
adenylate cyclase 2 [EC:4.6.1.1]
K08043  
adenylate cyclase 3 [EC:4.6.1.1]
K08044  
adenylate cyclase 4 [EC:4.6.1.1]
K08045  
adenylate cyclase 5 [EC:4.6.1.1]
K08046  
adenylate cyclase 6 [EC:4.6.1.1]
K08047  
adenylate cyclase 7 [EC:4.6.1.1]
K08048  
adenylate cyclase 8 [EC:4.6.1.1]
K08049  
adenylate cyclase 9 [EC:4.6.1.1]
K04345  
protein kinase A [EC:2.7.11.11]
K04850  
voltage-dependent calcium channel L type alpha-1C
K04851  
voltage-dependent calcium channel L type alpha-1D
K04853  
voltage-dependent calcium channel L type alpha-1F
K04857  
voltage-dependent calcium channel L type alpha-1S
K04862  
voltage-dependent calcium channel beta-1
K04863  
voltage-dependent calcium channel beta-2
K04864  
voltage-dependent calcium channel beta-3
K04865  
voltage-dependent calcium channel beta-4
K04858  
voltage-dependent calcium channel alpha-2/delta-1
K04859  
voltage-dependent calcium channel alpha-2/delta-2
K04860  
voltage-dependent calcium channel alpha-2/delta-3
K04861  
voltage-dependent calcium channel alpha-2/delta-4
K04866  
voltage-dependent calcium channel gamma-1
K04867  
voltage-dependent calcium channel gamma-2
K04868  
voltage-dependent calcium channel gamma-3
K04869  
voltage-dependent calcium channel gamma-4
K04870  
voltage-dependent calcium channel gamma-5
K04871  
voltage-dependent calcium channel gamma-6
K04872  
voltage-dependent calcium channel gamma-7
K04873  
voltage-dependent calcium channel gamma-8
K04962  
ryanodine receptor 2
K05852  
phospholamban
K05459  
insulin-like growth factor 1
K13375  
transforming growth factor beta-1
K13376  
transforming growth factor beta-2
K13377  
transforming growth factor beta-3
K03156  
tumor necrosis factor superfamily, member 2
Compound
C00076  
Calcium cation
C00575  
3',5'-Cyclic AMP
C01330  
Sodium cation
C02135  
Angiotensin II
Reference
  Authors
Fatkin D, Graham RM
  Title
Molecular mechanisms of inherited cardiomyopathies.
  Journal
Physiol Rev 82:945-80 (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)
Reference
  Authors
Zhao P, Sharma AC, Ren J
  Title
Pathogenesis and therapy of autoimmunity-induced dilated cardiomyopathy.
  Journal
Front Biosci 14:1708-15 (2009)
Reference
  Authors
Franz WM, Muller OJ, Katus HA
  Title
Cardiomyopathies: from genetics to the prospect of treatment.
  Journal
Lancet 358:1627-37 (2001)
Reference
  Authors
Towbin JA, Bowles NE
  Title
The failing heart.
  Journal
Nature 415:227-33 (2002)
Reference
  Authors
Luk A, Ahn E, Soor GS, Butany J
  Title
Dilated cardiomyopathy: a review.
  Journal
J Clin Pathol 62:219-25 (2009)
Reference
  Authors
Yoshikawa T, Baba A, Nagatomo Y
  Title
Autoimmune mechanisms underlying dilated cardiomyopathy.
  Journal
Circ J 73:602-7 (2009)
Reference
  Authors
Mason JW
  Title
Myocarditis and dilated cardiomyopathy: an inflammatory link.
  Journal
Cardiovasc Res 60:5-10 (2003)
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)
Reference
  Authors
Liu PP, Mason JW
  Title
Advances in the understanding of myocarditis.
  Journal
Circulation 104:1076-82 (2001)
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)

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