Below is an instruction from Human. Write a response.
    ### Instruction:
    What are the genetic changes related to beta-ketothiolase deficiency ?
    ### Response:
    Mutations in the ACAT1 gene cause beta-ketothiolase deficiency. This gene provides instructions for making an enzyme that is found in the energy-producing centers within cells (mitochondria). This enzyme plays an essential role in breaking down proteins and fats from the diet.
    