What are porphyrins in the body?

Porphyrins are ring-shaped molecules that undergo a series of chemical changes to produce heme — an important component of the protein hemoglobin, which carries oxygen in red blood cells.

What enzyme causes porphyria?

Acute intermittent porphyria (AIP) is a rare metabolic disorder that is characterized by partial deficiency of the enzyme hydroxymethylbilane synthase (also known as porphobilinogen deaminase). This enzyme deficiency can result in the accumulation of porphyrin precursors in the body.

What do high porphyrins mean?

This test looks for chemicals called porphyrins in your urine. If you have high levels of these chemicals, you may have porphyria. Porphyria refers to a group of inherited or acquired diseases that prevent your body from correctly making heme, the pigment in red blood cells that contains iron.

What can cause elevated porphyrins?

Factors that can trigger PCT include:

• Smoking.
• Drinking alcohol.
• HIV.
• Chronic hepatitis C.
• High iron levels in the body.
• Medications containing estrogens.
• Genetic changes resulting from hemochromatosis, a disease causing excessive iron absorption in the body.

What is porphyrins in chemistry?

Porphyrins (/ˈpɔːrfərɪn/ POR-fər-in) are a group of heterocyclic macrocycle organic compounds, composed of four modified pyrrole subunits interconnected at their α carbon atoms via methine bridges (=CH−). The parent of porphyrin is porphine, a rare chemical compound of exclusively theoretical interest.

What are porphyrins in skin?

Porphyrins are intermediate metabolites in the biosynthesis of vital molecules, including heme, cobalamin, and chlorophyll. Bacterial porphyrins are known to be proinflammatory, with high levels linked to inflammatory skin diseases.

What are porphyrins in urine?

Porphyrins are natural chemicals in the body that help form many important substances in the body. One of these is hemoglobin, the protein in red blood cells that carries oxygen in the blood. Porphyrins can be measured in the urine or blood. This article discusses the urine test.

How are porphyrins excreted?

The porphyrins that occur in the first half of the heme synthetic pathway (see Fig. 112-1) are water soluble and are excreted in urine.

Why is urine red in porphyria?

The terms porphyrin and porphyria are derived from the Greek word porphyrus, meaning purple. Urine from porphyria patients may be dark or reddish in color due to the presence of excess porphyrins and related substances, and may darken further after exposure to light.

What is porphyrin in urine?

Definition. Porphyrins are natural chemicals in the body that help form many important substances in the body. One of these is hemoglobin, the protein in red blood cells that carries oxygen in the blood. Porphyrins can be measured in the urine or blood .

What causes elevated porphyrins in urine?

Increases of urine porphyrins are found with congenital erythropoietic porphyria, acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and porphyria cutanea tarda. Fecal porphyrin examination for hereditary coproporphyria, variegate porphyria, and protoporphyria can be used for adult patients.