Monomethyl fumarate, sold under the brand name Bafiertam is a medication for the treatment of relapsing forms of multiple sclerosis, to include clinically isolated syndrome, relapsing-remitting disease, and active secondary progressive disease, in adults. It is taken by mouth. The most common adverse reactions are flushing, abdominal pain, diarrhea, and nausea.It was approved for medical use in the United States in April 2020. Pharmacology
Monomethyl fumarate alters the NFE2L2 (Nuclear factor erythroid 2-related factor 2) transcription factor. NFE2L2 (or NRF2) is a basic leucine zipper (bZIP) protein that regulates the expression of antioxidant proteins that protect against oxidative damage triggered by injury and inflammation. Several drugs that stimulate the NFE2L2 pathway are being studied for treatment of diseases that are caused by oxidative stress. Two precursors are also approved:

Dimethyl fumarate (brand name Tecfidera),
Diroximel fumarate (brand name Vumerity), approved by the FDA in October 2019. Society and culture
Names
Monomethyl fumarate is the international nonproprietary name (INN). References
External links
"Monomethyl fumarate". Drug Information Portal. U.S. National Library of Medicine.
The enzyme is involved in the last step of producing tetrahydrobiopterin, better known as BH4. BH4 is involved in the processing of amino acids and the production of neurotransmitters, specifically that of dopamine and serotonin which are primarily used in transmission of signals between nerve cells in the brain. The mutation in the SPR gene interferes with the production of the enzyme by producing enzymes with little or no function at all. This interference results in a lack of BH4 specifically in the brain. The lack of BH4 only occurs in the brain because other parts of the body adapt and utilize alternate pathways for the production of BH4. The mutation in the SPR gene leads to nonfunctional sepiapterin reductase enzymes, which results in a lack of BH4 and ultimately disrupts the production of dopamine and serotonin in the brain. The disruption of dopamine and serotonin production leads to the visible symptoms present in patients suffering from sepiapterin reductase deficiency. SR deficiency is considered an inherited autosomal recessive condition disorder because each parent carries one copy of the mutated gene, but typically do not show any signs or symptoms of the condition. Diagnosis
CSF neurotransmitter screening
The diagnosis of SR deficiency is based on the analysis of the pterins and biogenic amines found in the cerebrospinal fluid (CSF) of the brain. The pterin compound functions as a cofactor in enzyme catalysis and biogenic amines which include adrenaline, dopamine, and serotonin have functions that vary from the control of homeostasis to the management of cognitive tasks.