Entry |
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Name |
Dilated cardiomyopathy - Loxodonta africana (African savanna elephant)
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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.
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Class |
Human Diseases; Cardiovascular disease
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Pathway map |

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Organism |
Loxodonta africana (African savanna elephant) [GN: lav]
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Gene |
100660968 | TNNC1; troponin C, slow skeletal and cardiac muscles isoform X1 [KO:K05865] |
100656664 | LOW QUALITY PROTEIN: myosin-binding protein C, fast-type [KO:K12568] |
100654973 | ADRB1; LOW QUALITY PROTEIN: beta-1 adrenergic receptor [KO:K04141] |
100655206 | guanine nucleotide-binding protein G(s) subunit alpha isoform X1 [KO:K04632] |
100659992 | CACNA1D; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851] |
100674649 | CACNA1F; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853] |
100673089 | CACNA1S; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857] |
104846285 | CACNB1; voltage-dependent L-type calcium channel subunit beta-1 isoform X1 [KO:K04862] |
100673091 | CACNB2; voltage-dependent L-type calcium channel subunit beta-2 [KO:K04863] |
100653996 | CACNB3; voltage-dependent L-type calcium channel subunit beta-3 isoform X4 [KO:K04864] |
100656938 | CACNB4; voltage-dependent L-type calcium channel subunit beta-4 [KO:K04865] |
100676022 | CACNA2D1; voltage-dependent calcium channel subunit alpha-2/delta-1 [KO:K04858] |
100667110 | CACNA2D2; voltage-dependent calcium channel subunit alpha-2/delta-2 [KO:K04859] |
100659138 | CACNA2D3; LOW QUALITY PROTEIN: voltage-dependent calcium channel subunit alpha-2/delta-3 [KO:K04860] |
100663604 | CACNA2D4; voltage-dependent calcium channel subunit alpha-2/delta-4 [KO:K04861] |
100666105 | CACNG1; voltage-dependent calcium channel gamma-1 subunit [KO:K04866] |
100672366 | CACNG3; voltage-dependent calcium channel gamma-3 subunit [KO:K04868] |
100664775 | CACNG4; voltage-dependent calcium channel gamma-4 subunit [KO:K04869] |
100665821 | CACNG5; voltage-dependent calcium channel gamma-5 subunit isoform X2 [KO:K04870] |
111749631 | CACNG6; voltage-dependent calcium channel gamma-6 subunit [KO:K04871] |
100672020 | CACNG7; voltage-dependent calcium channel gamma-7 subunit [KO:K04872] |
100660460 | CACNG8; voltage-dependent calcium channel gamma-8 subunit [KO:K04873] |
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Compound |
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Reference |
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Authors |
Fatkin D, Graham RM |
Title |
Molecular mechanisms of inherited cardiomyopathies. |
Journal |
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Reference |
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Authors |
Lappe JM, Pelfrey CM, Tang WH |
Title |
Recent insights into the role of autoimmunity in idiopathic dilated cardiomyopathy. |
Journal |
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Reference |
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Authors |
Zhao P, Sharma AC, Ren J |
Title |
Pathogenesis and therapy of autoimmunity-induced dilated cardiomyopathy. |
Journal |
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Reference |
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Authors |
Franz WM, Muller OJ, Katus HA |
Title |
Cardiomyopathies: from genetics to the prospect of treatment. |
Journal |
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Reference |
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Authors |
Towbin JA, Bowles NE |
Title |
The failing heart. |
Journal |
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Reference |
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Authors |
Luk A, Ahn E, Soor GS, Butany J |
Title |
Dilated cardiomyopathy: a review. |
Journal |
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Reference |
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Authors |
Yoshikawa T, Baba A, Nagatomo Y |
Title |
Autoimmune mechanisms underlying dilated cardiomyopathy. |
Journal |
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Reference |
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Authors |
Mason JW |
Title |
Myocarditis and dilated cardiomyopathy: an inflammatory link. |
Journal |
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Reference |
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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 |
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Reference |
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Authors |
Liu PP, Mason JW |
Title |
Advances in the understanding of myocarditis. |
Journal |
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Reference |
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Authors |
Tam PE |
Title |
Coxsackievirus myocarditis: interplay between virus and host in the pathogenesis of heart disease. |
Journal |
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Related pathway |
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KO pathway |
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LinkDB |
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