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Related Concept Videos

Overview of Fatty Acid Metabolism01:28

Overview of Fatty Acid Metabolism

Lipids also are sources of energy that power cellular processes. Like carbohydrates, lipids are composed of carbon, hydrogen, and oxygen, but these atoms are arranged differently. Most lipids are nonpolar and hydrophobic. Major types include fats and oils, waxes, phospholipids, and steroids.
Fatty acids are catabolized in a process called beta-oxidation, which takes place in the matrix of the mitochondria and converts their fatty acid chains into two-carbon units of acetyl groups. The acetyl...
Protein Import into the Peroxisomes01:27

Protein Import into the Peroxisomes

Cells contain membrane-bound organelles called peroxisomes that oxidize organic molecules by transferring hydrogen atoms to oxygen, producing hydrogen peroxide. Peroxisomes enzymatically convert the released hydrogen peroxide into water and oxygen.
Peroxisomal Protein Import:
Peroxisomes lack the genetic machinery required to code for their own proteins. Hence, most peroxisomal membrane, lumenal and transmembrane proteins are synthesized in the cytoplasm or ER and transported to the peroxisome...
Fats as Energy Storage Molecules01:06

Fats as Energy Storage Molecules

Triglycerides are a form of long-term energy storage molecules. They are made of glycerol and three fatty acids. To obtain energy from fat, triglycerides must first be broken down by hydrolysis into their two principal components, fatty acids and glycerol. This process, called lipolysis, takes place in the cytoplasm. The resulting fatty acids are oxidized by β-oxidation into acetyl-CoA, which is used by the Krebs cycle. The glycerol that is released from triglycerides after lipolysis directly...
Overview of Lipid Metabolism01:24

Overview of Lipid Metabolism

Lipid metabolism is a crucial process in the human body that involves the synthesis and degradation of lipids. This process is essential for energy production, cell membrane formation, and hormone production, among other functions.
Lipolysis: The Breakdown of Lipids:
Lipolysis is the process of breaking down lipids, particularly triglycerides, into glycerol and fatty acids. This process typically occurs in the adipose tissue and is triggered by various hormones, including glucagon and...
Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Lipid Catabolism01:25

Lipid Catabolism

Triglycerides serve as crucial long-term energy storage molecules in microorganisms, providing a dense source of metabolic energy. Their breakdown is mediated by lipases, which hydrolyze triglycerides into glycerol and free fatty acids. Each of these components follows distinct metabolic pathways, ultimately contributing to ATP synthesis and cellular energy homeostasis.Glycerol MetabolismGlycerol, released from triglyceride hydrolysis, is phosphorylated by glycerol kinase to form...

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Determination of Fatty Acid Oxidation and Lipogenesis in Mouse Primary Hepatocytes
12:11

Determination of Fatty Acid Oxidation and Lipogenesis in Mouse Primary Hepatocytes

Published on: August 27, 2015

Fatty acid oxidation disorders.

Piero Rinaldo1, Dietrich Matern, Michael J Bennett

  • 1Department of Laboratory Medicine and Pathology, Mayo Clinic and Foundation, Biochemical Genetics Laboratory, Rochester, Minnesota 55905, USA. rinaldo@mayo.edu

Annual Review of Physiology
|February 5, 2002
PubMed
Summary
This summary is machine-generated.

Genetic disorders of mitochondrial fatty acid beta-oxidation are significant causes of illness and death. This review details over 20 inherited diseases affecting this crucial energy pathway.

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Measurement of Fatty Acid &#946;-Oxidation in a Suspension of Freshly Isolated Mouse Hepatocytes
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Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle
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Measurement of Fatty Acid &#946;-Oxidation in a Suspension of Freshly Isolated Mouse Hepatocytes
11:03

Measurement of Fatty Acid β-Oxidation in a Suspension of Freshly Isolated Mouse Hepatocytes

Published on: September 9, 2021

Area of Science:

  • Biochemistry
  • Genetics
  • Molecular Biology

Background:

  • Mitochondrial fatty acid beta-oxidation is vital for energy production, but its mechanisms are still being uncovered.
  • Genetic defects in this pathway are increasingly recognized as major contributors to disease.
  • The understanding of this system has evolved from a simple four-step process to a complex, membrane-bound, and tissue-specific function.

Purpose of the Study:

  • To review the normal process of mitochondrial fatty acid beta-oxidation.
  • To discuss the clinical, metabolic, and molecular aspects of over 20 known inherited diseases of this pathway.
  • To highlight the physiological significance of fatty acids as an energy source.

Main Methods:

  • Review of existing literature on mitochondrial fatty acid beta-oxidation.
  • Analysis of clinical, metabolic, and molecular data from patients with inherited defects.
  • Synthesis of current knowledge regarding the enzymatic components and their localization.
  • Exploration of the genetic basis of these disorders, including single-gene and multifactorial influences.

Main Results:

  • Mitochondrial fatty acid beta-oxidation involves complex enzymatic machinery, with some enzymes located on the inner mitochondrial membrane.
  • Over 20 distinct inherited diseases affecting this pathway have been identified.
  • Disease phenotypes can arise from single-gene defects or combinations of genetic and environmental factors.
  • Tissue-specific enzyme expression influences the manifestation of these disorders.

Conclusions:

  • Inherited disorders of mitochondrial fatty acid beta-oxidation represent a significant group of diseases with diverse clinical presentations.
  • Continued research is essential to fully elucidate the intricacies of this metabolic pathway and its associated pathologies.
  • Understanding these genetic defects is crucial for diagnosis, management, and potential therapeutic strategies.