Heme arginate (or haem arginate) is a compound of
heme
Heme, or haem (pronounced / hi:m/ ), is a precursor to hemoglobin, which is necessary to bind oxygen in the bloodstream. Heme is biosynthesized in both the bone marrow and the liver.
In biochemical terms, heme is a coordination complex "consis ...
and
arginine used in the treatment of acute
porphyrias. This heme product is only available outside the
United States
The United States of America (U.S.A. or USA), commonly known as the United States (U.S. or US) or America, is a country primarily located in North America. It consists of 50 states, a federal district, five major unincorporated territori ...
and is equivalent to hematin.
Heme arginate is a heme compound, whereby
L-arginine is added to prevent rapid degradation. It is given intravenously, and its action of mechanism is to reduce the overproduction of
δ-aminolevulinic acid
δ-Aminolevulinic acid (also dALA, δ-ALA, 5ALA or 5-aminolevulinic acid), an endogenous non-proteinogenic amino acid, is the first compound in the porphyrin synthesis pathway, the pathway that leads to heme in mammals, as well as chlorophyll in ...
, which can cause the acute symptoms in an attack of the acute porphyrias.
See also
*
Acute intermittent porphyria
Acute intermittent porphyria (AIP) is a rare metabolic disorder affecting the production of heme resulting from a deficiency of the enzyme porphobilinogen deaminase. It is the most common of the acute porphyrias.
Signs and symptoms
The clinical ...
*
Aminolevulinic acid
*
Inborn error of metabolism
Inborn errors of metabolism form a large class of genetic diseases involving congenital disorders of enzyme activities. The majority are due to defects of single genes that code for enzymes that facilitate conversion of various substances ( substr ...
References
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Porphyrins