The failing heart

Towbin, Jeffrey A.; Bowles, Neil E.

doi:10.1038/415227a

Cited by 492 publications

(316 citation statements)

References 69 publications

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“…In summary, several reports confirm that CM hamsters with genetic loss of delta-sarcoglycan recapitulate many pathophysiologic aspects of cardiac failure [39, 267,296], but it is clear that despite its extended use, this model is far from being totally understood [247].…”

Section: Syrian Cardiomyopathic Hamstermentioning

confidence: 99%

Rodent models of heart failure: an updated review

et al. 2012

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Heart failure (HF) is one of the major health and economic burdens worldwide, and its prevalence is continuously increasing. The study of HF requires reliable animal models to study the chronic changes and pharmacologic interventions in myocardial structure and function and to follow its progression toward HF. Indeed, during the past 40 years, basic and translational scientists have used small animal models to understand the pathophysiology of HF and find more efficient ways of preventing and managing patients suffering from congestive HF (CHF). Each species and each animal model has advantages and disadvantages, and the choice of one model over another should take them into account for a good experimental design. The aim of this review is to describe and highlight the advantages and drawbacks of some commonly used HF rodents models, including both non-genetically and genetically engineered models, with a specific subchapter concerning diastolic HF models.

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Section: Syrian Cardiomyopathic Hamstermentioning

confidence: 99%

Rodent models of heart failure: an updated review

et al. 2012

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“…99 DCM is characterized by an increased ventricular chamber size and reduced systolic output. 57 RCM results from processes that stiffen the myocardium by infiltration or fibrosis leading to impaired ventricular filling and reduced diastolic volume of either or both ventricles with the cavity size, wall thickness of the ventricles and ejection remaining near normal.…”

Section: Cardiomyopathymentioning

confidence: 99%

Intermediate Filament Diseases: Desminopathy

Goldfarb

Olivé

Vicart³

et al. 2008

Advances in Experimental Medicine and Biology

104

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Desminopathy is one of the most common intermediate filament human disorders associated with mutations in closely interacting proteins, desmin and alphaB-crystallin. The inheritance pattern in familial desminopathy is characterized as autosomal dominant or autosomal recessive, but many cases have no family history. At least some and likely most sporadic desminopathy cases are associated with de novo DES mutations. The age of disease onset and rate of progression may vary depending on the type of inheritance and location of the causative mutation. Typically, the illness presents with lower and later upper limb muscle weakness slowly spreading to involve truncal, neckflexor, facial and bulbar muscles. Skeletal myopathy is often combined with cardiomyopathy manifested by conduction blocks, arrhythmias and chronic heart failure resulting in premature sudden death. Respiratory muscle weakness is a major complication in some patients. Sections of the affected skeletal and cardiac muscles show abnormal fibre areas containing chimeric aggregates consisting of desmin and other cytoskeletal proteins. Various DES gene mutations: point mutations, an insertion, small in-frame deletions and a larger exon-skipping deletion, have been identified in desminopathy patients. The majority of these mutations are located in conserved alpha-helical segments, but additional mutations have recently been identified in the tail domain. Filament and network assembly studies indicate that most but not all disease-causing mutations make desmin assembly-incompetent and able to disrupt a pre-existing filamentous network in dominant-negative fashion. AlphaBcrystallin serves as a chaperone for desmin preventing its aggregation under various forms of stress; mutant CRYAB causes cardiac and skeletal myopathies identical to those resulting from DES mutations.

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“…Cardiomyopathy is defined as a disease of the heart muscle that is associated with cardiac dysfunction (21 (25,26) and may be diagnosed using echocardiography (22,25,27 (21).…”

Section: Cardiomyopathymentioning

confidence: 99%