Isovaleric acidaemia is a relatively uncommon condition where the body cannot effectively degrade a specific protein-building component (amino acid). To put it another way, it is an organic acid disease, which is a problem that involves an abnormal accumulation of organic acids. Organo-acidemia, organo-aciduria, and organo-acidemia in the cells can all be hazardous and lead to significant health complications.
Amino acids are the minor components of proteins that the body dissolves typically from meals. Amino acids could be further metabolized as an energy source for progress and expansion. Individuals with isovaleric acidemia lack an enzyme that breaks down leucine, a specific amino acid.