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Biotinidase deficiency is an autosomal recessive metabolic disorder in which biotin is not released from proteins in the diet during digestion or from normal protein turnover in the cell. This situation results in biotin deficiency. Biotin is an important water-soluble nutrient that aids in the metabolism of fats, carbohydrates, and proteins.
Normally, the amount of biotin in the body is regulated by dietary intake, biotin transporters (monocarboxylate transporter 1 and sodium-dependent multivitamin transporter), peptidyl hydrolase biotinidase (BTD), and the protein ligase holocarboxylase synthetase. When any of these regulatory factors are inhibited, biotin deficiency could occur.
One-fourth cup of roasted almonds provides 1.5 mcg of biotin, which is 5% of your daily value. ... “Even a single dose of biotin can interfere with thyroid function tests within 24 hours of ...
[1] [36] In the US, biotin as a non-prescription dietary supplement is sold in amounts of 1 to 10 mg per serving, with claims for supporting hair and nail health, and as 300 mg per day as a possibly effective treatment for multiple sclerosis [37] [38] (see § Research). Overconsumption of 5 mg/day or higher causes elevated concentration in ...
Biotin, a B vitamin, is found in foods such as liver, egg yolks, and milk. It is essential for the normal production and breakdown of proteins, fats, and carbohydrates in the body. Mutations in the HLCS gene reduce the activity of holocarboxylase synthetase, preventing cells from using biotin effectively and disrupting many cellular functions.
Researchers believe there may be a link between long-term vitamin D deficiency and the development of autoimmune conditions, such as type 1 diabetes and rheumatoid arthritis, but more research is ...
How to Boost Energy Levels. Let’s take a look at some practical tips and strategies for how to have more energy. 1. Aim for a Balanced Diet. Maintaining balanced blood sugar levels is key to ...
Holocarboxylase synthetase (biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)), also known as protein—biotin ligase, is a family of enzymes (EC 6.3.4.10). This enzyme is important for the effective use of biotin, a B vitamin found in foods such as liver, egg yolks, and milk. In many of the body's tissues, holocarboxylase ...