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  2. Valine - Wikipedia

    en.wikipedia.org/wiki/Valine

    Valine (symbol Val or V) [4] is an α-amino acid that is used in the biosynthesis of proteins. It contains an α-amino group (which is in the protonated −NH 3 + form under biological conditions), an α-carboxylic acid group (which is in the deprotonated −COO − form under biological conditions), and a side chain isopropyl group, making it a non-polar aliphatic amino acid.

  3. Hartnup disease - Wikipedia

    en.wikipedia.org/wiki/Hartnup_disease

    Nicotinamide is necessary for neutral amino acid transporter production in the proximal renal tubules found in the kidney, and intestinal mucosal cells found in the small intestine. Therefore, a symptom stemming from this disorder results in increased amounts of amino acids in the urine.

  4. Hypervalinemia - Wikipedia

    en.wikipedia.org/wiki/Hypervalinemia

    Hypervalinemia is a rare autosomal recessive metabolic disorder in which urinary and serum levels of the branched-chain amino acid valine are elevated, without related elevation of the branched-chain amino acids leucine and isoleucine. [2] [3] It is caused by a deficiency of the enzyme valine transaminase. [4]

  5. Aminoaciduria - Wikipedia

    en.wikipedia.org/wiki/Aminoaciduria

    In renal aminoaciduria, the renal tubules are unable to reabsorb the filtered amino acids back into the blood, causing high concentrations of amino acids in the urine. [5] This may be caused by a defect in the transport proteins in the renal tubule, for example, as occurs in Hartnup disease , [ 5 ] or may be due to damage to the kidney tubule ...

  6. Intradialytic parenteral nutrition - Wikipedia

    en.wikipedia.org/wiki/Intradialytic_parenteral...

    It is administered directly into the bloodstream of patients with chronic kidney disease (CKD) in an effort to decrease the associated morbidity and mortality experienced in patients with kidney failure. IDPN contains protein (amino acids), carbohydrates , and fats in an attempt to meet a patient's weekly nutritional needs. Solutions can be ...

  7. Organic acidemia - Wikipedia

    en.wikipedia.org/wiki/Organic_acidemia

    Organic acidemia is a term used to classify a group of metabolic disorders which disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids which are usually not present. [1] The branched-chain amino acids include isoleucine, leucine and valine. [1]

  8. Maple syrup urine disease - Wikipedia

    en.wikipedia.org/wiki/Maple_syrup_urine_disease

    Maple syrup urine disease (MSUD) is a rare, inherited metabolic disorder that affects the body's ability to metabolize amino acids due to a deficiency in the activity of the branched-chain alpha-ketoacid dehydrogenase (BCKAD) complex. [3] It particularly affects the metabolism of amino acids—leucine, isoleucine, and valine. [4]

  9. Cachexia - Wikipedia

    en.wikipedia.org/wiki/Cachexia

    The branched-chain amino acids leucine and valine may have potential in inhibiting overexpression of protein breakdown pathways. [38] The amino acid glutamine has been used as a component of oral supplementation to reverse cachexia in people with advanced cancer [39] or HIV/AIDS. [40]

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