Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Porin Insertion in the Outer Mitochondrial Membrane

Porin Insertion in the Outer Mitochondrial Membrane

Porins are beta-barrel proteins translocated to the mitochondrial outer membrane through the TOM complex into the intermembrane space. Porin precursors bind TIM chaperones within the intermembrane space and are guided to the Sorting and Assembly Machinery complex or SAM complex on the outer mitochondrial membrane.
Three models describe the assembly of porins by the SAM complex and their insertion into the outer membrane. Model 1 suggests that porins are assembled outside the SAM channel as the...

Fates of Pyruvate

Fates of Pyruvate

Pyruvate is the end product of glycolysis, where glucose is oxidized to pyruvate, simultaneously reducing NAD+ to NADH. Two molecules of ATP are also produced by substrate-level phosphorylation.
In aerobic organisms, pyruvate is metabolized via the citric acid cycle to produce reduced coenzymes NADH and FADH2. These coenzymes are then oxidized in the electron transport chain to produce ATP and, in the process, regenerate the NAD+ and FAD. As seen in some cell types and organisms, fermentation...

Pyruvate Oxidation

Pyruvate Oxidation

After glycolysis, the charged pyruvate molecules enter the mitochondria via active transport and undergo three enzymatic reactions. These reactions ensure that pyruvate can enter the next metabolic pathway so that energy stored in the pyruvate molecules can be harnessed by the cells.
First, the enzyme pyruvate dehydrogenase removes the carboxyl group from pyruvate and releases it as carbon dioxide. The stripped molecule is then oxidized and releases electrons, which are then picked up by NAD+...

Corrosion

Corrosion

The degradation of metals due to natural electrochemical processes is known as corrosion. Rust formation on iron, tarnishing of silver, and the blue-green patina that develops on copper are examples of corrosion. Corrosion involves the oxidation of metals. Sometimes it is protective, such as the oxidation of copper or aluminum, wherein a protective layer of metal oxide or its derivatives forms on the surface, protecting the underlying metal from further oxidation. In other cases, corrosion is...

Peripheral Artery Disease I: Introduction

Peripheral Artery Disease I: Introduction

Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs but also impacts other areas, such as the arms, thereby impairing overall circulation and organ function.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty deposits inside the arterial...

Oxidation of Phenols to Quinones

Oxidation of Phenols to Quinones

In the presence of oxidizing agents, phenols are oxidized to quinones. Quinones can be easily reduced back to phenols using mild reducing agents. The electron-donating hydroxyl group enhances the reactivity of the aromatic ring, enabling oxidation of the ring even in the absence of an α hydrogen.
o-hydroxy phenols are oxidized to o-quinones and p-hydroxy phenols to p-quinones. Such redox reactions involve the transfer of two electrons and two protons. The reversible redox...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Complementary and alternative medicines and cannabis use among individuals with acute intermittent porphyria.

Molecular genetics and metabolism·2026

Same author

Accessibility and harmonization of biochemical tests for diagnosis and monitoring of Porphyrias in the United States: Recommendations by members of the American Porphyrias Expert Collaborative (APEX).

Molecular genetics and metabolism·2026

Same author

Age-Specific Parkinson Disease Risk in Gaucher Disease Type 1: Data From the ICGG Gaucher Registry.

Neurology·2026

Same author

Complementary and alternative medicines and cannabis use among individuals with erythropoietic protoporphyria.

Molecular genetics and metabolism·2025

Same author

Bitopertin shows efficacy in patients with erythropoietic protoporphyria: Results from the randomized, double-blind, placebo-controlled AURORA trial.

Journal of the American Academy of Dermatology·2025

Same author

Immune thrombocytopenia in patients treated with immune checkpoint inhibitors.

Blood·2025

Search research articles

Related Experiment Video

Updated: Jul 20, 2025

Percutaneous Hepatic Perfusion PHP with Melphalan as a Treatment for Unresectable Metastases Confined to the Liver

Percutaneous Hepatic Perfusion PHP with Melphalan as a Treatment for Unresectable Metastases Confined to the Liver

Published on: July 31, 2016

Update on the Porphyrias.

Amy K Dickey^1,2, Rebecca Karp Leaf^2,3, Manisha Balwani⁴

¹Division of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA;

Annual Review of Medicine

|August 4, 2023

Summary

This summary is machine-generated.

The porphyrias are rare genetic diseases caused by enzyme defects in heme production. Early diagnosis and management are crucial for preventing complications from these multisystemic disorders.

Keywords:

acute hepatic porphyria acute intermittent porphyria congenital erythropoietic porphyria erythropoietic protoporphyria porphyria porphyria cutanea tarda protoporphyria

More Related Videos

Author Spotlight: Porphyrin-Modified Beads for Use as Compensation Controls in Flow Cytometry

Author Spotlight: Porphyrin-Modified Beads for Use as Compensation Controls in Flow Cytometry

Published on: March 24, 2023

One Minute, Sub-One-Watt Photothermal Tumor Ablation Using Porphysomes, Intrinsic Multifunctional Nanovesicles

One Minute, Sub-One-Watt Photothermal Tumor Ablation Using Porphysomes, Intrinsic Multifunctional Nanovesicles

Published on: September 17, 2013

Related Experiment Videos

Last Updated: Jul 20, 2025

Percutaneous Hepatic Perfusion PHP with Melphalan as a Treatment for Unresectable Metastases Confined to the Liver

Percutaneous Hepatic Perfusion PHP with Melphalan as a Treatment for Unresectable Metastases Confined to the Liver

Published on: July 31, 2016

Author Spotlight: Porphyrin-Modified Beads for Use as Compensation Controls in Flow Cytometry

Author Spotlight: Porphyrin-Modified Beads for Use as Compensation Controls in Flow Cytometry

Published on: March 24, 2023

One Minute, Sub-One-Watt Photothermal Tumor Ablation Using Porphysomes, Intrinsic Multifunctional Nanovesicles

One Minute, Sub-One-Watt Photothermal Tumor Ablation Using Porphysomes, Intrinsic Multifunctional Nanovesicles

Published on: September 17, 2013

Area of Science:

Biochemistry
Genetics
Internal Medicine

Background:

Porphyrias are rare genetic disorders stemming from enzymatic defects in the heme biosynthetic pathway.
These diseases are classified as hepatic or erythropoietic based on heme intermediate accumulation.
They present as multisystemic conditions with diverse clinical manifestations.

Purpose of the Study:

To provide a comprehensive review of the porphyrias.
To describe their clinical presentation, diagnostic approaches, and management strategies.
To discuss emerging and approved novel therapies.

Main Methods:

Literature review of porphyria research.
Analysis of clinical presentation, diagnosis, and management.
Review of current and developing therapeutic options.

Main Results:

Porphyrias result from specific enzyme deficiencies in heme synthesis.
Symptoms vary widely and affect multiple organ systems.
Effective management relies on early detection and tailored treatment.

Conclusions:

Porphyrias require a multidisciplinary approach for diagnosis and management.
Prompt identification is essential to mitigate long-term health consequences.
Novel therapeutic strategies offer improved treatment prospects.