Document Detail

Metabolic cardiomyopathies.
MedLine Citation:
PMID:  11298185     Owner:  NLM     Status:  MEDLINE    
The energy needed by cardiac muscle to maintain proper function is supplied by adenosine Ariphosphate primarily (ATP) production through breakdown of fatty acids. Metabolic cardiomyopathies can be caused by disturbances in metabolism, for example diabetes mellitus, hypertrophy and heart failure or alcoholic cardiomyopathy. Deficiency in enzymes of the mitochondrial beta-oxidation show a varying degree of cardiac manifestation. Aberrations of mitochondrial DNA lead to a wide variety of cardiac disorders, without any obvious correlation between genotype and phenotype. A completely different pathogenetic model comprises cardiac manifestation of systemic metabolic diseases caused by deficiencies of various enzymes in a variety of metabolic pathways. Examples of these disorders are glycogen storage diseases (e.g. glycogenosis type II and III), lysosomal storage diseases (e.g. Niemann-Pick disease, Gaucher disease, I-cell disease, various types of mucopolysaccharidoses, GM1 gangliosidosis, galactosialidosis, carbohydrate-deficient glycoprotein syndromes and Sandhoff's disease). There are some systemic diseases which can also affect the heart, for example triosephosphate isomerase deficiency, hereditary haemochromatosis, CD 36 defect or propionic acidaemia.
B Guertl; C Noehammer; G Hoefler
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  International journal of experimental pathology     Volume:  81     ISSN:  0959-9673     ISO Abbreviation:  Int J Exp Pathol     Publication Date:  2000 Dec 
Date Detail:
Created Date:  2001-04-12     Completed Date:  2001-05-10     Revised Date:  2013-06-11    
Medline Journal Info:
Nlm Unique ID:  9014042     Medline TA:  Int J Exp Pathol     Country:  England    
Other Details:
Languages:  eng     Pagination:  349-72     Citation Subset:  IM    
Institute of Pathology, University of Graz, Austria.
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MeSH Terms
Calcium / metabolism
Cardiomegaly / metabolism
Cardiomyopathies / etiology*,  metabolism*
Cardiomyopathy, Alcoholic / metabolism
Carnitine / deficiency
Diabetes Mellitus / metabolism
Fatty Acids / metabolism
Glucose / metabolism
Heart Failure / metabolism
Lysosomal Storage Diseases / metabolism
Malonyl Coenzyme A / metabolism
Mitochondrial Myopathies / metabolism
Mucopolysaccharidoses / metabolism
Myocardium / metabolism*
Oxidative Phosphorylation
Reg. No./Substance:
0/Fatty Acids; 50-99-7/Glucose; 524-14-1/Malonyl Coenzyme A; 541-15-1/Carnitine; 7440-70-2/Calcium

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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