Mitochondrial trifunctional protein deficiency

Mitochondrial trifunctional protein deficiency
Other namesTFP deficiency
Mitochondrial trifunctional protein deficiency has an autosomal recessive pattern of inheritance
SymptomsCardiomyopathy, skeletal myopathy
TypesMutations in the HADHA and HADHB gene
Diagnostic methodCBC, Urine test
TreatmentLow fat diet, Limited exercise

Mitochondrial trifunctional protein deficiency (MTP deficiency or MTPD) is an autosomal recessive fatty acid oxidation disorder that prevents the body from converting certain fats to energy, particularly during periods without food.

People with this disorder have inadequate levels of an enzyme that breaks down a certain group of fats called long-chain fatty acids.