From Wikipedia, the free encyclopedia
Carnitine-acylcarnitine translocase (CACT) is responsible for passive transport of carnitine and carnitine- fatty acid complexes and across the inner mitochondrial membrane as part of the carnitine shuttle system.
Function [ edit ]
Fatty acyl–carnitine can diffuse from the cytosol across the porous outer mitochondrial membrane to the intermembrane space, but must utilize CACT to cross the nonporous inner mitochondrial membrane and reach the mitochondrial matrix. CACT is a
cotransporter, returning one molecule of carnitine from the matrix to the intermembrane space as one molecule of fatty acyl–carnitine moves into the matrix.
Clinical significance [ edit ]
A disorder is associated with
carnitine-acylcarnitine translocase deficiency. This disorder disrupts the carnitine shuttle system from moving fatty acids across the mitochondrial membrane, leading to a decrease in fatty acid catabolism. The result is an accumulation of fatty acid within muscles and liver, decreased tolerance to long term exercise, inability to fast for more than a few hours, muscle weakness and wasting, and a strong acidic smell on the breath (due to protein catabolism).
Acyl-CoA from cytosol to the mitochondrial matrix
Model organisms [ edit ]
Model organisms have been used in the study of SLC25A20 function. A conditional knockout mouse line called Slc25a20 was generated at the tm1a(EUCOMM)Wtsi Wellcome Trust Sanger Institute. Male and female animals underwent a standardized  phenotypic screen to determine the effects of deletion.     Additional screens performed: - In-depth immunological phenotyping 
Slc25a20 knockout mouse phenotype
All data available at. 
Peripheral blood leukocytes 6 Weeks
6 Weeks Haematology Normal
Homozygous viability at P14
Recessive lethal study Normal
Indirect calorimetry Abnormal
Glucose tolerance test Normal
Auditory brainstem response Normal
Clinical chemistry Normal
16 Weeks Haematology Normal
Peripheral blood leukocytes 16 Weeks
infection Salmonella Normal
Cytotoxic T Cell Function
Mesenteric Lymph Node Immunophenotyping
Bone Marrow Immunophenotyping
Epidermal Immune Composition
References [ edit ]
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