Electrocardiographic and biochemical abnormalities in Tay-Sachs disease

Rodríguez‐Torres, Ramón; Schneck, Larry; Kleinberg, William

doi:10.1016/0002-9149(69)90338-5

Cited by 5 publications

(6 citation statements)

References 1 publication

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“…Patients with Tay-Sachs disease have no clinical manifestations of cardiovascular disease but show nonspecific electrocardiographic changes and accumulations in heart tissue of a GM 2 ganglioside similar to that found in excessive amounts in the brain [54].…”

Section: Gangliosidosismentioning

confidence: 97%

“…Two types of GM 2 gangliosidosis include Tay-Sachs disease (type I) with deficiency of hexosamini- dase A (hex A), and Sandhoff disease (type II) with deficiency of hex A and ␤-N-acetyl hexosaminidase. Patients with Tay-Sachs disease have no clinical manifestations of cardiovascular disease but show nonspecific electrocardiographic changes and accumulations in heart tissue of a GM 2 ganglioside similar to that found in excessive amounts in the brain [54].…”

Section: Gangliosidosismentioning

confidence: 97%

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Nonmalformative Cardiovascular Pathology in Infants and Children

Gilbert‐Barness

Barness

1999

Pediatr Dev Pathol

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Section: Gangliosidosismentioning

confidence: 97%

Section: Gangliosidosismentioning

confidence: 97%

Nonmalformative Cardiovascular Pathology in Infants and Children

Gilbert‐Barness

Barness

1999

Pediatr Dev Pathol

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“…Patients with Tay-Sachs disease have no clinical manifestations of cardiovascular disease but show nonspeci¢c electrocardiographic changes and accumulations in heart tissue of a GM 2 ganglioside similar to that found in excessive amounts in the brain [45].…”

Section: Gangliosidosismentioning

confidence: 88%

Cardiovascular Involvement in Metabolic Diseases

Gilbert‐Barness

2002

Pediatric Pathology & Molecular Medicine

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Cardiomyopathies are a group of disorders that result in dysfunction of the contractility of the myocardium anda decrease in cardiac function. Cardiomyopathies may be primary or idiopathic or secondary due to an underlying definable systemic disorder. Cardiomyopathies may be further defined as according to the World Health Organization (WHO) as: 1) congestive or dilated; 2) obstructive as in hypertrophic cardiomyopathy; 3) restrictive as in infiltrative disorders such as storage diseases. Two additional categories have been added that include arrhythmogenic right ventricular cardiomyopathy and unclassified cardiomyopathies. A number of metabolic disorders result in cardiomyopathy and are frequently the cause of death in these disorders.

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“…Patients with Tay-Sachs disease have accumulations of GM3/GD3 gangliosides, mainly in the brain and rarely in the heart. 97 98 In Sandhoff disease, also called GM2 gangliosidosis, the β-galactosidase A and B enzymes are deficient, leading to accumulation of GM3/GD3 gangliosides. The GM2-ganglioside storage disorders are heritable diseases, which are essentially neurodegenerative diseases of early infancy.…”

Section: Sphingolipids In Secondary Cardiomyopathiesmentioning

confidence: 99%

Sphingolipid Mediators of Myocardial Pathology

Kovilakath

Cowart

2020

J Lipid Atheroscler

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Cardiomyopathy is the leading cause of mortality worldwide. While the causes of cardiomyopathy continue to be elucidated, current evidence suggests that aberrant bioactive lipid signaling plays a crucial role as a component of cardiac pathophysiology. Sphingolipids have been implicated in the pathophysiology of cardiovascular disease, as they regulate numerous cellular processes that occur in primary and secondary cardiomyopathies. Experimental evidence gathered over the last few decades from both in vitro and in vivo model systems indicates that inhibitors of sphingolipid synthesis attenuate a variety of cardiomyopathic symptoms. In this review, we focus on various cardiomyopathies in which sphingolipids have been implicated and the potential therapeutic benefits that could be gained by targeting sphingolipid metabolism.

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Electrocardiographic and biochemical abnormalities in Tay-Sachs disease

Cited by 5 publications

References 1 publication

Nonmalformative Cardiovascular Pathology in Infants and Children

Nonmalformative Cardiovascular Pathology in Infants and Children

Cardiovascular Involvement in Metabolic Diseases

Sphingolipid Mediators of Myocardial Pathology

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