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Most adults can take 500 mg daily for up to 6 months without monitoring

While we could demonstrate evidence of L-carnitine deficiency and accumulation of -BB as direct consequences of gbh-1/BBOX1 deficiency, the absence of significant changes in other metabolites confirms that the loss of GBH-1 selectively impacts the L-carnitine biosynthesis pathway without significantly affecting other metabolic pathways in amino acid, nucleotide, and energy metabolism that are not dependent on L-carnitine (Fig

However, in some serious cases the patient may require a blood transfusion or dialysis as treatment

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Improved oxygen delivery: Vitamin B12 is needed for the production of red blood cells, which carry oxygen to the muscles

Another benefit of IV therapy is that it can be customized to specific needs