3-Hydroxyisovalerate

3-Hydroxyisovalerate is an organic acid produced during the breakdown of branched-chain amino acids (proteins). Elevated levels in urine can indicate metabolic disorders affecting amino acid metabolism, such as maple syrup urine disease or other organic acidemias. This urine test helps detect inherited metabolic conditions that may cause developmental delays, neurological problems, or nutritional deficiencies if left untreated. It is often part of newborn screening or evaluation for unexplained metabolic symptoms.