Methylenetetrahydrofolate reductase (MTHFR) is an enzyme required for the formation of 5-methyltetrahydrofolate (5-MTHF), a form of folate able to cross the blood-brain barrier and which is necessary as a substrate for the remethylation of homocysteine to methionine by methionine synthase. Patients with severe MTHFR deficiency cannot make 5-MTHF and have extremely low levels in the CSF. Only treatment with oral 5-MTHF given as calcium mefolinate resulted in an increase in CSF 5-MTHF.

Estriol (E3), also spelled oestriol, is a steroid, a weak agonist of the estrogen receptors ERα and ERβ., and a minor female sex hormone. According to in vitro study, the relative binding affinity (RBA) of estriol for the human ERα and ERβ was 11.3% and 17.6% of that estradiol, respectively, and the relative transactivational capacity of estrone at the ERα and ERβ was 10.6% and 16.6% of that of estradiol, respectively. Estriol is marketed widely in Europe and elsewhere throughout the world under the brand names Ovestin, Ortho-Gynest, and a variety of others. It is available in oral tablet, vaginal cream, and vaginal suppository form, and is used in menopausal hormone therapy for the treatment of menopausal symptoms. Estriol is also available in some countries as estriol succinate (brand name Synapause), a dosage-equivalent ester prodrug of estriol. Estriol and estriol succinate are not approved for use in the United States and Canada, although they have been produced and sold by compounding pharmacies in North America for use as a component of bioidentical hormone therapy. Estriol can be measured in maternal blood or urine and can be used as a marker of fetal health and well-being. If levels of unconjugated estriol (uE3 or free estriol) are abnormally low in a pregnant woman, this may indicate chromosomal or congenital anomalies like Down syndrome or Edward's syndrome. It is included as part of the triple test and quadruple test for antenatal screening for fetal anomalies.

Flavin adenine dinucleotide is a coenzyme form of vitamin B2. Many oxidoreductases, called flavoenzymes or flavoproteins, require FAD as a prosthetic group, which functions in electron transfers. It is usually used for the prevention and treatment of various diseases that are caused by Vitamin B2 deficiency or metabolic disorder including stomatitis, eczema, etc. No adverse reactions were reported.

D-mannose is a simple sugar found naturally in fruits such as cranberries and pineapples. Unlike many sugars, it is not metabolised or stored in the liver. Much of it is excreted in the urine, where it interferes with particle attachment and prevents certain kind of bacteria from sticking to the walls and causing infection. Mannose supplement is also indicated for treatment of carbohydrate-deficient glycoprotein syndrome, however clinical trials failed to prove its efficacy.

Ornithine is an amino acid produced in the urea cycle by the splitting off of urea from arginine. It is a central part of the urea cycle, which allows for the disposal of excess nitrogen. Ornithine is also a precursor of citrulline and arginine. Arginine stimulates the pituitary release of growth hormone. Burns or other injuries affect the state of arginine in tissues throughout the body. As de novo synthesis of arginine during these conditions is usually not sufficient for normal immune function, nor for normal protein synthesis, ornithine may have immunomodulatory and wound-healing activities under these conditions (by virtue of its metabolism to arginine).

Behenic acid is a saturated fatty acid that is derived from the oil extracts of plants and used as a component of conditioning agents. Behenic acid is also a part of a novel complex of lipophilic ingredients developed for the treatment of dry skin. The properties of behenic acid were studied in comparison to others fatty acids and it was found that behenic acid does not inhibit the UDP-glucuronosyltransferase (UGT) 1A1 enzyme. The high levels of behenic acid in patients with low-grade glial tumors is an important indicator of the persistence of tissue integrity and tissue resistance. Therefore, behenic acid levels can be a prognostic factor in glial tumors.

L-carnitine (L-beta-hydroxy-gamma-N,N,N-trimethylaminobutyric acid) is conditionally necessary for mitochondrial transport and metabolism of long-chain fatty acids, and thus for myocardial energetic metabolism. D-carnitine is not biologically active and might interfere with proper utilization of the L isomer, and so there are claims that the racemic mixture (DL-carnitine) should be avoided. The pharmacological effects of carnitine are stereospecific: L-carnitine was effective in various animal and clinical studies, while D- and DL-carnitine was found to be ineffective or even toxic to some cells and tissues, such as muscle cells and the myocardium. DL-carnitine caused symptoms of myasthenia and cardiac arrhythmias, which disappeared after L-carnitine administration.

Adenosine triphosphate (ATP) is an adenine nucleotide containing three phosphate groups esterified to the sugar moiety. Adenosine triphosphate is the energy source in living cells. In physiological conditions, the average concentration varies from 3150 mM in mammalian cells to 1500–1900 mM in human blood cells. Extracellular adenosine and adenosine triphosphate (ATP) are involved in biological processes including neurotransmission, muscle contraction, cardiac function, platelet function, vasodilatation, signal transduction and secretion in a variety of cell types. A large family of membrane-bound receptors mediates cell signalling by ATP and adenosine. These purinergic receptors ultimately determine the variety of effects induced by extracellular ATP and adenosine. ATP and adenosine have strong negative chronotropic and dromotropic effects on the mammalian heart. The sensitivity of the sinus node and the atrioventricular node to ATP and adenosine manifests pronounced variability among species. For more than three decades, ATP has been used routinely in Europe in the acute therapy of paroxysmal supraventricular tachycardia. ATPace™, an injectable formulation of adenosine 5′-triphosphate (ATP), was developed by Cordex Pharma, Inc. (Cordex) as a diagnostic and therapeutic drug for the management of cardiac bradyarrhythmias. Extracellular ATP exerts multiple effects in various cell types by activating cell-surface receptors known as P2 receptors. In the heart, ATP suppresses the automaticity of cardiac pacemakers and atrioventricular (AV) nodal conduction via adenosine, the product of its degradation by ecto-enzymes, as well as by triggering a cardio-cardiac vagal reflex. ATP, given as a rapid intravenous bolus injection, has been used since the late 1940s as a highly effective and safe therapeutic agent for the acute termination of reentrant paroxysmal supraventricular tachycardia (PSVT) involving the AV node. In addition, preliminary studies have shown that ATP can also be used as a diagnostic agent for the identification of several cardiac disorders including sinus node dysfunction (sick sinus syndrome), dual AV nodal pathways, long QT syndrome, and bradycardic syncope. The US Food and Drug Administration has approved Cordex formulation for ATP as an Investigational New Drug and two pathways for its marketing approval; one therapeutic, i.e., acute termination of paroxysmal PSVT, and the other diagnostic, i.e., the identification of patients with bradycardic syncope who can benefit from pacemaker therapy. However later ATPace development for the treatment of bradycardia and paroxysmal supraventricular tachycardia was discontinued.