Aminoglow Oral

Indications

• Urea cycle disorders
• Hyperammonaemia types I and II
• Citrullinaemia
• Arginosuccinic aciduria
• Deficiency of N-acetyl glutamate synthetase
• Acute hyperammonaemia in carbamylphosphate synthetase deficiency
• Acute hyperammonaemia in ornithine transcarbamylase deficiency

Dosage

Children: Neonate: 100–300 mg/kg daily in 3–4 divided doses; Child (body-weight up to 40 kg): 6 mg/kg/hour; Child (body-weight 40 kg and above): 4 mg/kg/hour.

Adults: Refer to BNF for specific dosing based on condition; typically administered as 5–50 mg/kg twice daily, adjusted according to plasma ammonia concentration.

Mechanism of action

Arginine serves as a substrate for the synthesis of nitric oxide (NO), a crucial signaling molecule, and contributes to the urea cycle, facilitating the detoxification of ammonia in the liver.

Pharmacodynamics

As a precursor for nitric oxide, arginine plays a vital role in vasodilation and modulating blood flow. It also aids in protein synthesis and supports metabolic processes in the body.

Pharmacokinetics

Arginine is absorbed from the gastrointestinal tract and is distributed throughout the body. It undergoes hepatic metabolism, primarily via the urea cycle, and its half-life is influenced by renal function.

Pregnancy

Use only if clearly needed; consult specialist.

Breast-feeding

Use with caution; benefits should outweigh risks.

Storage

Store in a cool, dry place away from direct sunlight.

Formulations

• L-Arginine 500 mg tablets
• L-Arginine 1 gram tablets
• L-Arginine powder for solution
• L-Arginine 500 mg capsules
• L-Arginine 21% concentrate for solution for infusion

Indications

• Nutritional supplementation
• Support for muscle metabolism
• Enhancement of immune function

Dosage

Children: Refer to the BNF for Children for appropriate paediatric dosing guidelines.

Adults: Refer to the BNF for specific dosing information as it varies based on clinical indications.

Mechanism of action

L-Alanine is synthesized from pyruvate via transamination. It serves as an energy source for muscle cells and is significant in the Alanine-Glucose cycle, where it is converted to glucose in the liver. This process is pivotal for regulating blood sugar levels, especially during extended exercise, when branched-chain amino acids (BCAAs) are used for energy and their nitrogen portions are utilized to form L-Alanine.

Pharmacodynamics

L-Alanine is an important energy source for muscle tissue, the brain, and the central nervous system. It strengthens the immune system by facilitating the production of antibodies and plays a role in the metabolism of sugars and organic acids, which are essential for various physiological functions.

Pharmacokinetics

L-Alanine is readily available in the plasma as a free amino acid. It is involved in several metabolic pathways, including biotin metabolism and tRNA charging. The metabolism of L-Alanine primarily occurs in the liver, where it can be converted into glucose, thus participating in the body's energy homeostasis.

Pregnancy

There are no known specific contraindications for the use of alanine during pregnancy; however, it should be used under medical supervision.

Breast-feeding

There is limited data on the effects of alanine during breastfeeding, and it is advised to consult a healthcare provider before use.

Storage

Store in a cool, dry place away from direct sunlight and moisture.

Formulations

• L-Alanine powder
• L-Alanine capsules
• L-Alanine tablets

Indications

• Nutritional supplementation
• Support in metabolic disorders
• Potential role in improving cognitive function
• Used in sports nutrition for muscle recovery

Dosage

Children: Refer to specific product information or guidelines for appropriate dosing as it can vary based on the formulation and clinical indication.

Adults: Refer to specific product information or guidelines for appropriate dosing as it can vary based on the formulation and clinical indication.

Mechanism of action

Aspartic acid acts as an excitatory neurotransmitter in the central nervous system by stimulating the N-methyl-D-aspartate (NMDA) receptors and other glutamate receptors. This action contributes to neuronal excitability, synaptic plasticity, and cognitive functions such as learning and memory.

Pharmacodynamics

Aspartic acid influences neuronal activity through its role as a neurotransmitter, modulating synaptic transmission and neuroplasticity. It is also involved in the biosynthesis of other amino acids and neurotransmitters, which further impacts various physiological processes, including hormone secretion and energy metabolism.

Pharmacokinetics

Aspartic acid is readily absorbed from the gastrointestinal tract following oral administration. It is distributed widely throughout the body, where it participates in metabolic processes. The metabolism of aspartic acid involves transamination to form oxaloacetate, which enters the Krebs cycle. Excretion primarily occurs through the kidneys as part of the urea cycle.

Pregnancy

There is limited data on the safety of aspartic acid during pregnancy. Consult healthcare providers for guidance.

Breast-feeding

Limited information is available on the excretion of aspartic acid in breast milk. Consult healthcare providers before use.

Storage

Store in a cool, dry place away from direct sunlight. Keep out of reach of children.

Indications

• Oxidative stress conditions
• Wound healing
• Mucolytic therapy in respiratory diseases such as bronchitis and cystic fibrosis
• Support in vitamin B6 utilization

Dosage

Children: Refer to BNF for Children for specific dosing recommendations.

Adults: Refer to BNF for specific dosing recommendations.

Mechanism of action

L-Cystine acts as a precursor for glutathione synthesis, which is essential for cellular protection against oxidative stress, particularly in conditions like acetaminophen overdose where glutathione levels are depleted.

Pharmacodynamics

L-Cystine is formed by the oxidation of two cysteine molecules and is involved in several physiological processes. It contributes to the synthesis of glutathione, which helps in detoxifying reactive oxygen species and maintaining cellular health. Cystine also aids in the healing of wounds and burns, supports insulin supply to the pancreas, and may enhance the breakdown of mucus in conditions such as bronchitis and cystic fibrosis.

Pharmacokinetics

Cystine is absorbed in the gastrointestinal tract and utilized in various metabolic pathways. It is subject to transformation in the body, contributing to cysteine levels and subsequently to glutathione production. The excretion of cystine occurs primarily through the kidneys, where it can form calculi if concentrations become excessively high.

Pregnancy

There is insufficient evidence to determine the safety of cystine during pregnancy. Consult a healthcare professional for advice.

Breast-feeding

Cystine is generally considered safe during breastfeeding, but it is advisable to consult a healthcare professional before use.

Storage

Store in a cool, dry place, away from direct sunlight. Keep out of reach of children.

Formulations

• Cystine powder for oral solution
• Cystine tablets

Mechanism of action

Many of supplemental L-arginine's activities, including its possible anti-atherogenic actions, may be accounted for by its role as the precursor to nitric oxide or NO. NO is produced by all tissues of the body and plays very important roles in the cardiovascular system, immune system and nervous system. NO is formed from L-arginine via the enzyme nitric oxide synthase or synthetase (NOS), and the effects of NO are mainly mediated by 3,'5' -cyclic guanylate or cyclic GMP. NO activates the enzyme guanylate cyclase, which catalyzes the synthesis of cyclic GMP from guanosine triphosphate or GTP. Cyclic GMP is converted to guanylic acid via the enzyme cyclic GMP phosphodiesterase. NOS is a heme-containing enzyme with some sequences similar to cytochrome P-450 reductase. Several isoforms of NOS exist, two of which are constitutive and one of which is inducible by immunological stimuli. The constitutive NOS found in the vascular endothelium is designated eNOS and that present in the brain, spinal cord and peripheral nervous system is designated nNOS. The form of NOS induced by immunological or inflammatory stimuli is known as iNOS. iNOS may be expressed constitutively in select tissues such as lung epithelium. All the nitric oxide synthases use NADPH (reduced nicotinamide adenine dinucleotide phosphate) and oxygen (O2) as cosubstrates, as well as the cofactors FAD (flavin adenine dinucleotide), FMN (flavin mononucleotide), tetrahydrobiopterin and heme. Interestingly, ascorbic acid appears to enhance NOS activity by increasing intracellular tetrahydrobiopterin. eNOS and nNOS synthesize NO in response to an increased concentration of calcium ions or in some cases in response to calcium-independent stimuli, such as shear stress. In vitro studies of NOS indicate that the Km of the enzyme for L-arginine is in the micromolar range. The concentration of L-arginine in endothelial cells, as well as in other cells, and in plasma is in the millimolar range. What this means is that, under physiological conditions, NOS is saturated with its L-arginine substrate. In other words, L-arginine would not be expected to be rate-limiting for the enzyme, and it would not appear that supraphysiological levels of L-arginine which could occur with oral supplementation of the amino acid^would make any difference with regard to NO production. The reaction would appear to have reached its maximum level. However, in vivo studies have demonstrated that, under certain conditions, e.g. hypercholesterolemia, supplemental L-arginine could enhance endothelial-dependent vasodilation and NO production.

Pharmacodynamics

Studies have shown that is has improved immune responses to bacteria, viruses and tumor cells; promotes wound healing and regeneration of the liver; causes the release of growth hormones; considered crucial for optimal muscle growth and tissue repair.

Mechanism of action

L-Alanine is a non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases immunity, and provides energy for muscle tissue, brain, and the central nervous system. BCAAs are used as a source of energy for muscle cells. During prolonged exercise, BCAAs are released from skeletal muscles and their carbon backbones are used as fuel, while their nitrogen portion is used to form another amino acid, Alanine. Alanine is then converted to Glucose by the liver. This form of energy production is called the Alanine-Glucose cycle, and it plays a major role in maintaining the body's blood sugar balance.

Pharmacodynamics

Is an important source of energy for muscle tissue, the brain and central nervous system; strengthens the immune system by producing antibodies; helps in the metabolism of sugars and organic acids.

Mechanism of action

Certain conditions, e.g. an acetaminophen overdose, deplete hepatic glutathione and subject the tissues to oxidative stress resulting in loss of cellular integrity. L-Cystine serves as a major precursor for synthesis of glutathione.

Pharmacodynamics

L-Cystine is a covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. Cystine is a chemical substance which naturally occurs as a deposit in the urine, and can form a calculus (hard mineral formation) when deposited in the kidney. The compound produced when two cysteine molecules linked by a disulfide (S-S) bond. Cystine is required for proper vitamin B6 utilization and is also helpful in the healing of burns and wounds, breaking down mucus deposits in illnesses such as bronchitis as well as cystic fibrosis. Cysteine also assists in the supply of insulin to the pancreas, which is needed for the assimilation of sugars and starches. It increases the level of glutathione in the lungs, liver, kidneys and bone marrow, and this may have an anti-aging effect on the body by reducing age-spots etc.