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Protein Import into the Peroxisomes

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...

Peroxisomes

Peroxisomes

Peroxisomes are specialized organelles present in fungi, plant, and animal cells. It can vary in number, size, morphology, and activity depending on the type of tissue and the nutritional state of the cell. For example, cells with active lipid metabolism, such as adipocytes, neurons, and hepatocytes, have more peroxisomes than other cells in the body. Besides their primary role in breaking down complex organic molecules, peroxisomes can also synthesize specific macromolecules and participate in...

Peroxisomes

Peroxisomes

Peroxisomes are specialized organelles present in fungi, plant, and animal cells. It can vary in number, size, morphology, and activity depending on the type of tissue and the nutritional state of the cell. For example, cells with active lipid metabolism, such as adipocytes, neurons, and hepatocytes, have more peroxisomes than other cells in the body. Besides their primary role in breaking down complex organic molecules, peroxisomes can also synthesize specific macromolecules and participate in...

Peroxisomes

Peroxisomes

Peroxisomes and mitochondria are two important oxygen-utilizing organelles in eukaryotic cells. Mitochondria carry out cellular respiration—the process that converts energy from food into ATP. Peroxisomes carry out a variety of functions, primarily breaking down different substances, such as fatty acids.The peroxisome is a single membrane-bound cellular organelle that can perform several different functions, including lipid metabolism and chemical detoxification. The enzymes within peroxisomes...

Inborn Errors of Metabolism

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...

Phase I Reactions: Oxidation of Aliphatic and Aromatic Carbon-Containing Systems

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Phase I biotransformation reactions are integral to drug metabolism, predominantly involving oxidative, reductive, and hydrolytic transformations. Chief among these are oxidative reactions, which enhance the hydrophilicity of xenobiotics and introduce polar functional groups to facilitate their elimination from the body.
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Related Experiment Video

Updated: Jul 10, 2026

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

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

Published on: September 9, 2021

Peroxisomal disorders affecting phytanic acid alpha-oxidation: a review.

A S Wierzbicki¹

¹Department of Chemical Pathology, St. Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, U.K. anthony.wierzbicki@kcl.ac.uk

Biochemical Society Transactions

|October 25, 2007

Summary

Peroxisomes manage fatty acid metabolism through alpha-, beta-, and omega-oxidation pathways. Research into these pathways clarifies diseases and suggests new cancer and Refsum

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Area of Science:

Biochemistry
Cell Biology
Medical Genetics

Background:

Peroxisomes are crucial for fatty acid synthesis and degradation.
They house key enzymes for alpha-, beta-, and omega-oxidation pathways.
Dysfunctional fatty acid metabolism is linked to various genetic disorders.

Purpose of the Study:

To elucidate the role of peroxisomal fatty acid oxidation in disease pathophysiology.
To investigate the involvement of specific enzymes like PhyH and AMACR.
To explore the connection between peroxisomal pathways and cancer progression.

Main Methods:

Enzyme activity assays.
Genetic analysis of patients with peroxisomal disorders.
Analysis of gene expression in cancer tissues.

Main Results:

Mutations in fatty acid degradation enzymes cause Refsum's disease, rhizomelic chondrodysplasia, and AMACR deficiency.
Fe(II)- and 2-oxoglutarate-dependent oxygenases (PhyH), thiamin-dependent lyases, and CYP4A are key players.
AMACR and other peroxisomal enzymes are highly expressed in prostate and renal cell cancers.

Conclusions:

Understanding peroxisomal fatty acid metabolism offers insights into genetic diseases.
Novel therapeutic strategies for Refsum's disease neuro-ophthalmological issues are emerging.
Peroxisomal pathways are implicated in obesity- and insulin resistance-related cancers.