Aplicação químico-farmacêutica da carnitina palmitoiltransferase-2 (CPT-II) através da regulação mitocondrial contra células tumorais cancerígenas

Background: Carnitine palmitoyltransferase II deficiency is an inherited disorder of long-chain fatty acid oxidation characterized by hypoketotic hypoglycemia, cardiomyopathy, seizures, muscle pain and weakness, and myoglobin. Individuals with carnitine palmitoyltransferase II deficiency have a defect in the production of the enzyme carnitine palmitoyltransferase-II, which plays an important role in fatty acid oxidation. Signs and symptoms of carnitine palmitoyltransferase II deficiency are due to the buildup of fatty acids and long-chain acyl-carnitine as well as reduced energy production in cells. Carnitine palmitoyltransferase II deficiency is an autosomal recessive disease caused by mutations in the CPT2 gene. During changing Nonalcoholic fatty liver disease (NAFLD) to the cirrhosis, the probability of cancer is high that should be considered as a dangerous situation. Methods: SDS-PAGE system of polyacrylamide gel electrophoresis through analytical method for separating charged molecules in mitochondrial mixtures according to their molecular mass in the presence of electrical fields was used. The Invitrogen® Bright Imaging (IBI) system provides was applied for the imaging and analysis of protein imprints. Results: Since currently no effective treatment for CPT-II deficiency, prevention of liver failure is a proper way of treatment through controlling mitochondria without affecting CPT-II potency. We discussed about the severe infantile hepatocardiac muscular position of CPT II deficiency affects the liver heart, and muscles. Conclusions: Through this work, we discussed and characterized the pathophysiological function in several tissues such as liver, Kidney cancers.

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Magazine Portal Bibliotech Digital (Universidad Nacional de Colombia)
Published
2025-03-17
Primary Topic
Metabolism and Genetic Disorders
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article

Aplicação químico-farmacêutica da carnitina palmitoiltransferase-2 (CPT-II) através da regulação mitocondrial contra células tumorais cancerígenas

Majid Monajjemi, Sara Shahriari, Samira Mohammadi, Fatemeh Mollaamin
Magazine Portal Bibliotech Digital (Universidad Nacional de Colombia)
Metabolism and Genetic Disorders
article

Aplicação químico-farmacêutica da carnitina palmitoiltransferase-2 (CPT-II) através da regulação mitocondrial contra células tumorais cancerígenas

Majid Monajjemi, Sara Shahriari, Samira Mohammadi, Fatemeh Mollaamin
article en

Abstract

Background: Carnitine palmitoyltransferase II deficiency is an inherited disorder of long-chain fatty acid oxidation characterized by hypoketotic hypoglycemia, cardiomyopathy, seizures, muscle pain and weakness, and myoglobin. Individuals with carnitine palmitoyltransferase II deficiency have a defect in the production of the enzyme carnitine palmitoyltransferase-II, which plays an important role in fatty acid oxidation. Signs and symptoms of carnitine palmitoyltransferase II deficiency are due to the buildup of fatty acids and long-chain acyl-carnitine as well as reduced energy production in cells. Carnitine palmitoyltransferase II deficiency is an autosomal recessive disease caused by mutations in the CPT2 gene. During changing Nonalcoholic fatty liver disease (NAFLD) to the cirrhosis, the probability of cancer is high that should be considered as a dangerous situation. Methods: SDS-PAGE system of polyacrylamide gel electrophoresis through analytical method for separating charged molecules in mitochondrial mixtures according to their molecular mass in the presence of electrical fields was used. The Invitrogen® Bright Imaging (IBI) system provides was applied for the imaging and analysis of protein imprints. Results: Since currently no effective treatment for CPT-II deficiency, prevention of liver failure is a proper way of treatment through controlling mitochondria without affecting CPT-II potency. We discussed about the severe infantile hepatocardiac muscular position of CPT II deficiency affects the liver heart, and muscles. Conclusions: Through this work, we discussed and characterized the pathophysiological function in several tissues such as liver, Kidney cancers.

Magazine Portal Bibliotech Digital (Universidad Nacional de Colombia)
Islamic Azad University Central Tehran Branch (IR), Kastamonu University (TR)
Affordable and clean energy
Openalex Percentile: Top 25%
Metabolism and Genetic Disorders
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Aplicação químico-farmacêutica da carnitina palmitoiltransferase-2 (CPT-II) através da regulação mitocondrial contra células tumorais cancerígenas — Majid Monajjemi, Sara Shahriari, et al. · Magazine Portal Bibliotech Digital (Universidad Nacional de Colombia) (2025) | TGRS Research Map | TGRS