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Heme arginate + L-arginine

Radboud University Medical Center · FDA-approved active Small molecule

Heme arginate replenishes depleted heme pools while L-arginine provides substrate for nitric oxide synthesis, together improving endothelial function and reducing vasoconstriction in acute porphyria crises.

Heme arginate replenishes depleted heme pools while L-arginine provides substrate for nitric oxide synthesis, together improving endothelial function and reducing vasoconstriction in acute porphyria crises. Used for Acute intermittent porphyria (acute attack management), Variegate porphyria (acute attack management), Hereditary coproporphyria (acute attack management).

At a glance

Generic nameHeme arginate + L-arginine
SponsorRadboud University Medical Center
Drug classHeme replacement therapy with nitric oxide donor
TargetALA synthase (indirect inhibition via heme feedback); nitric oxide synthase (L-arginine substrate)
ModalitySmall molecule
Therapeutic areaMetabolic/Genetic disorders
PhaseFDA-approved

Mechanism of action

In acute intermittent porphyria and related acute porphyrias, heme deficiency triggers overproduction of toxic porphyrin precursors. Heme arginate (a heme-albumin complex) directly replenishes heme and suppresses ALA synthase, the rate-limiting enzyme in porphyrin synthesis. L-arginine augments this effect by serving as a substrate for nitric oxide synthase, promoting vasodilation and reducing the hypertensive and vasoconstrictor complications characteristic of acute porphyria attacks.

Approved indications

Common side effects

Key clinical trials

Primary sources

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SourceUsed for
ClinicalTrials.govTrial enrolment, design, endpoints, results

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