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BIOTIN
- Biotin-thiamine-responsive basal ganglia disease is a disorder that affects the nervous system, including a group of structures in the brain called the basal ...
- ... enzyme is important for the effective use of biotin, a B vitamin found in foods such as ... tissues, holocarboxylase synthetase turns on (activates) enzymes called biotin-dependent carboxylases by attaching biotin to them. These ...
- ... the body is unable to use the vitamin biotin effectively. This disorder is classified as a multiple ... impaired activity of certain enzymes that depend on biotin.The signs and symptoms of holocarboxylase synthetase deficiency ...
- ... the body is unable to recycle the vitamin biotin. If this condition is not recognized and treated, ... making an enzyme called biotinidase. This enzyme recycles biotin, a B vitamin found in foods such as ...
- ... a region for binding to the B vitamin biotin, which is required for the enzyme to function. ... methylcrotonyl-CoA carboxylase alpha 3-methylcrotonyl-CoA carboxylase biotin-containing subunit MCCA MCCase subunit alpha MCCCalpha MCCCα ...
- ... CoA carboxylase 2 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit 3-methylcrotonyl-CoA carboxylase, beta MCCase ...
- The PC gene provides instructions for making an enzyme called pyruvate carboxylase. This enzyme is active in mitochondria, which are the energy-producing ...
- ... making an enzyme called biotinidase. This enzyme recycles biotin, a B vitamin found in foods such as liver, egg yolks, and milk. Biotinidase removes biotin that is bound to proteins in food, leaving ...
- ... SLC19A3 gene have been identified in people with biotin-thiamine-responsive basal ganglia disease, a disorder that ... disorders whose signs and symptoms overlap those of biotin-thiamine-responsive basal ganglia disease (described above). These ...
- ... a region for binding to the B vitamin biotin.Propionyl-CoA carboxylase plays a role in the ... cholesterol to a molecule called propionyl-CoA. Using biotin, propionyl-CoA carboxylase then converts propionyl-CoA to ...