Branched chain Ketonuria the mechanism of hypoglycemia in maple syrup urine disease

In maple syrup urine disease which is branched chain ketonuria there is hypoglycemia and it was once thought that the deficiency of leucine is the causative agent however now it is thought that it is the production of alanine and it's non availability for a gluconeogenic substrate which is responsible for hyperglycemia and hence chloric deprivation