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A review of fatty acid oxidation disorder mouse models.
Babcock, Shannon J; Houten, Sander M; Gillingham, Melanie B.
Afiliação
  • Babcock SJ; Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR, USA. Electronic address: babcocsh@ohsu.edu.
  • Houten SM; Deparment of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
  • Gillingham MB; Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR, USA.
Mol Genet Metab ; 142(1): 108351, 2024 May.
Article em En | MEDLINE | ID: mdl-38430613
ABSTRACT
Fatty acid oxidation disorders (FAODs) are a family of rare, genetic disorders that affect any part of the fatty acid oxidation pathway. Patients present with severe phenotypes, such as hypoketotic hypoglycemia, cardiomyopathy, and rhabdomyolysis, and currently manage these symptoms by the avoidance of fasting and maintaining a low-fat, high-carbohydrate diet. Because knowledge about FAODs is limited due to the small number of patients, rodent models have been crucial in learning more about these disorders, particularly in studying the molecular mechanisms involved in different phenotypes and in evaluating treatments for patients. The purpose of this review is to present the different FAOD mouse models and highlight the benefits and limitations of using these models. Specifically, we discuss the phenotypes of the available FAOD mouse models, the potential molecular causes of prominent FAOD phenotypes that have been studied using FAOD mouse models, and how FAOD mouse models have been used to evaluate treatments for patients.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Oxirredução / Modelos Animais de Doenças / Ácidos Graxos / Erros Inatos do Metabolismo Lipídico Limite: Animals / Humans Idioma: En Revista: Mol Genet Metab Assunto da revista: BIOLOGIA MOLECULAR / BIOQUIMICA / METABOLISMO Ano de publicação: 2024 Tipo de documento: Article País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Oxirredução / Modelos Animais de Doenças / Ácidos Graxos / Erros Inatos do Metabolismo Lipídico Limite: Animals / Humans Idioma: En Revista: Mol Genet Metab Assunto da revista: BIOLOGIA MOLECULAR / BIOQUIMICA / METABOLISMO Ano de publicação: 2024 Tipo de documento: Article País de publicação: Estados Unidos