NPL ANABOLIC WHEY POWDER (Each 43g serving contains dietary fibre/total sodium/Vitamin B6/Magnesium/Phosphorus/Potassium/Zinc/Taurine/L-glycine/Creatine monohydrate/ZMA 1.2g/379mg/0.5mg/49.3mg/170.7mg/128.9mg/1.4mg/3000mg/2500mg/2500mg/100mg)

Indications

• Homocystinuria
• Type I Gaucher's disease (specialist use only)

Dosage

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

Adults: Refer to BNF for specific dosing guidelines.

Mechanism of action

Betaine acts as a methyl donor in the transsulfuration pathway, facilitating the conversion of homocysteine to methionine and thereby reducing homocysteine levels.

Pharmacodynamics

Betaine reduces plasma homocysteine concentrations, which is beneficial in conditions such as homocystinuria. It also has a role in osmoregulation and may influence cellular metabolism.

Pharmacokinetics

Betaine is well absorbed after oral administration, with a bioavailability of approximately 90%. It is primarily excreted in the urine as betaine and its metabolites.

Adverse effects

• abdominal discomfort
• agitation
• alopecia
• appetite decreased
• brain oedema
• diarrhoea
• glossitis
• irritability
• nausea
• skin reactions
• urinary incontinence
• vomiting
• arthralgia
• asthenia
• chest discomfort
• dizziness
• dyspnoea
• fever
• flushing
• gastrointestinal discomfort
• headache
• hypersensitivity
• hypertension
• hypotension
• infusion-related reactions
• nausea
• pain
• skin reactions
• tachycardia

Interactions

• may be given with supplements of Vitamin B12, pyridoxine, and folate under specialist advice

Precautions

• use in conjunction with dietary restrictions
• monitor plasma-methionine concentration before and during treatment
• interrupt treatment if symptoms of cerebral oedema occur
• use with caution in pregnancy due to limited information
• use with caution in breastfeeding due to lack of information

Pregnancy

Manufacturer advises avoid unless essential-limited information available.

Breast-feeding

Manufacturer advises caution-no information available.

Storage

Store in a cool, dry place; protect from light.

Formulations

• Cystadane oral powder 1 gram per 1 gram

Indications

• Enhancement of athletic performance
• Improvement of exercise capacity
• Reduction of muscle fatigue during high-intensity exercise

Dosage

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

Adults: Refer to the BNF for specific dosing recommendations.

Mechanism of action

Beta-alanine acts as a precursor to carnosine, which is synthesized from beta-alanine and histidine within the muscle tissue. Carnosine functions as a pH buffer, helping to maintain optimal pH levels in muscle cells during exercise. This buffering action reduces the accumulation of hydrogen ions, which are responsible for muscle fatigue.

Pharmacodynamics

The primary pharmacodynamic effect of beta-alanine is its ability to increase muscle carnosine levels. Elevated carnosine concentrations enhance the muscle's capacity to buffer acid, thereby improving exercise performance, particularly in anaerobic conditions. The effects of beta-alanine supplementation are often observed as increased endurance and reduced perceived exertion during high-intensity activities.

Pharmacokinetics

Beta-alanine is rapidly absorbed from the gastrointestinal tract, with peak plasma concentrations typically reached within 30 to 60 minutes after ingestion. The elimination half-life of beta-alanine is relatively short, necessitating multiple doses throughout the day for optimal effects. It is metabolized in the liver and predominantly excreted in the urine. The bioavailability is considered high, with minimal first-pass metabolism.

Pregnancy

There is insufficient data on the safety of beta-alanine in pregnancy. Consult relevant guidelines.

Breast-feeding

Beta-alanine is not well-studied in breastfeeding. Caution is advised when using this supplement.

Storage

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

Indications

• Supplementation for athletes to enhance performance in high-intensity, short-duration exercises
• Potential use in neurodegenerative diseases for its neuroprotective properties
• Cardioprotective applications in certain cardiac conditions

Dosage

Children: Refer to BNF for Children for specific pediatric dosing guidelines.

Adults: Refer to BNF for specific dosing recommendations as they can vary based on individual circumstances and conditions.

Mechanism of action

In the muscles, some creatine binds to phosphate, forming creatine phosphate, catalyzed by creatine kinase (CK). Phosphocreatine then interacts with adenosine diphosphate (ADP) to regenerate ATP, supplying energy for short-term needs before oxidative phosphorylation becomes dominant. This process is particularly important during anaerobic exercise, where energy supply shifts from oxidative to anaerobic pathways.

Pharmacodynamics

Creatine is synthesized from L-arginine, glycine, and L-methionine and is transported to various tissues including skeletal muscle, heart, and brain. In these tissues, creatine is predominantly converted into phosphocreatine, serving as a major energy storage form. The energy-generating capacity of supplemental creatine during high-intensity exercise is coupled with potential neuroprotective and cardioprotective effects.

Pharmacokinetics

Creatine is rapidly absorbed from the gastrointestinal tract following oral administration. It is distributed primarily to skeletal muscle, where it is stored as phosphocreatine. The half-life of creatine in plasma is approximately 3 hours, and it is excreted mainly through the kidneys as creatinine, a breakdown product.

Adverse effects

• Gastrointestinal discomfort
• Diarrhea
• Nausea
• Muscle cramping
• Weight gain due to water retention

Precautions

• Use with caution in individuals with pre-existing kidney disease or renal impairment.
• Monitor renal function periodically during prolonged use.

Pregnancy

There is insufficient data on the safety of creatine supplementation during pregnancy. It is recommended to avoid use unless prescribed by a healthcare professional.

Breast-feeding

Creatine supplementation is not well studied in breastfeeding women. Use with caution and consult a healthcare provider.

Storage

Store in a cool, dry place, away from direct sunlight. Keep container tightly closed.

Formulations

• Powder
• Capsules
• Tablets

Indications

• Nutritional supplementation in adults and children
• Management of conditions related to taurine deficiency
• Support in metabolic disorders

Dosage

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

Adults: Refer to BNF for specific dosing guidelines.

Mechanism of action

Taurine functions by replacing missing nutrients in the body. It serves as a substrate for bile salt formation, regulates cell volume, modulates intracellular calcium levels, and provides cytoprotection in the central nervous system.

Pharmacodynamics

Taurine supplements are well-tolerated and serve as a nitrogen source for nutritional support. The administration of taurine regulates plasma amino acid concentrations, nitrogen balance, weight, and serum protein levels to normalize values and enhance overall nutritional status.

Pharmacokinetics

Taurine is absorbed from the gastrointestinal tract and is distributed widely in the body, particularly in tissues with high metabolic activity. It is excreted primarily through urine. The pharmacokinetics may vary based on dietary intake and individual metabolic rates.

Pregnancy

Taurine is generally considered safe during pregnancy, but consult a healthcare provider before use.

Breast-feeding

Taurine is thought to be safe during breastfeeding, but it is advisable to consult a healthcare provider.

Storage

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

Formulations

• Dietary supplements containing taurine

Mechanism of action

Homocystinuria is a hereditary disorder characterized by high levels of the amino acid homocysteine. This condition can be caused by deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylenetetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (cbl). CBS converts homocysteine to cystathionine, and a deficiency in this enzyme can lead to an accumulation of homocysteine. MTHFR is responsible for producing 5-methyltetrahydrofolate, a methyl donor that participates in the conversion of homocysteine back to methionine; therefore, an MTHFR deficiency can also lead to homocystinuria. Additionally, a defect in cobalamin (vitamin B12) cofactor metabolism can lead to homocystinuria, since a metabolite of cobalamin (methylcobalamin) promotes the conversion of homocysteine to methionine. Betaine transfers a methyl group via the enzyme betaine homocysteine methyl transferase (BHMT), converting homocysteine back into methionine and dimethylglycine (DMG). In patients with homocystinuria, betaine reduces homocysteine levels and improves health outcomes. Betaine acts as metabolic intermediate in transmethylating processes (creatine and methionine synthesis). Betaine acts as a methyl group donor in the remethylation of homocysteine to methionine in patients with homocystinuria. This reduces toxic concentrations of homocysteine, usually to 20 to 30% or less of pretreatment concentrations. Betaine or trimethylglycine is a quarternary ammonium compound that was first discovered in the juice of sugar beets (Beta vulgaris). Betaine is a metabolite of choline ... and is a substrate in one of the two recycling pathways that convert homocysteine to L-methionine. The other and principal recycling reaction is catalyzed by the enzyme methionine synthase and uses methylcobalamin as a cofactor and 5-methyltetrahydrofolate as a cosubstrate. Betaine-homocysteine methyltransferase (BHMT) is a zinc metalloenzyme which catalyzes the transfer of a methyl group from betaine to homocysteine in the formation of methionine. BHMT is found in the liver and kidneys and may also exist in brain tissue. Betaine acts to lower homocysteine levels in some with primary hyperhomocysteinemia/homocystinuria via this enzyme.

Pharmacodynamics

Betaine decreases plasma homocysteine concentrations in homocystinuria cases caused by deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylenetetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (cbl). The decrease of homocysteine is estimated to be 20-30% of pre-treatment levels. Betaine supplementation in patients with homocystinuria also improves metabolic abnormalities in cerebrospinal fluid. Reports have shown that depending on the type of homocystinuria, the therapeutic effectiveness of betaine alone may be limited, insufficient to decrease total homocysteine levels and prevent clinical symptoms. In patients with homocystinuria due to cystathionine beta-synthase (CBS) deficiency, betaine should be used when serum total homocysteine levels remain high despite dietary therapy. Patients taking betaine for several years do not show evidence of tolerance. Also, betaine concentrations are not correlated with homocysteine concentrations. In patients with MTHFR deficiency and cbl defects, betaine may increase methionine and S-adenosyl methionine (SAM) plasma levels. Patients with CBS deficiency without a dietary restriction of methionine may accumulate excessive amounts of methionine. Clinical data shows that in patients with CBS deficiency, increased plasma methionine levels were associated with cerebral edema.

Mechanism of action

In the muscles, a fraction of the total creatine binds to phosphate - forming creatine phosphate. The reaction is catalysed by creatine kinase, and the result is phosphocreatine (PCr). Phosphocreatine binds with adenosine diphosphate to convert it back to ATP (adenosine triphosphate), an important cellular energy source for short term ATP needs prior to oxidative phosphorylation. Supplemental creatine may have an energy-generating action during anaerobic exercise and may also have neuroprotective and cardioprotective actions. Creatine, creatine kinase and phosphocreatine make up an intricate cellular enegy buffering and transport system connecting sites of energy production in the mitochondria with sites of energy consumption. CK is a key enzyme in involved in cellular energy homeostasis. It reversibly catalyzes the transfer of the high-energy phosphate bond in PCr to adenosine diphosphate (ADP) to form adenosine triphosphate (ATP), and it catalyzes the transfer of the high-energy phosphate bond in ATP to creatine to form PCr. During periods of intense exercise and skeletal muscle contraction, bioenergetic metabolism switches from one in which oxidative phosphorylation is the major pathway of ATP production to one in which so-called anaerobic glycolysis becomes dominant.

Pharmacodynamics

Creatine is a essential, non-proteinaceous amino acid derivative found in all animals. It is synthesized in the kidney, liver, and pancreas from L-arginine, glycine and L-methionine. Following its biosynthesis, creatine is transported to the skeletal muscle, heart, brain and other tissues. Most of the creatine is metabolized in these tissues to phosphocreatine (creatine phosphate). Phosphocreatine is a major energy storage form in the body. Supplemental creatine may have an energy-generating action during anaerobic exercise and may also have neuroprotective and cardioprotective actions.

Mechanism of action

The diet supplements containing taurine function by replacing the missing nutriments in the body. Taurine, as a single agent, presents different functions like substrate for formation of bile salts, cell volume regulation, modulation of intracellular calcium, cytoprotection of central nervous system, etc.

Pharmacodynamics

The diet supplements containing taurine are formulated as a well-tolerated nitrogen source for nutritional support. Administration of diet supplements regulates the level of plasma amino acid concentration, nitrogen balance, weight and serum protein concentration to reach normal values, thus improving the nutritional status.