SLC25A6
Description
The SLC25A6 (solute carrier family 25 member 6) is a protein-coding gene located on chromosome X|Y.
ADP/ATP translocase 3, also known as solute carrier family 25 member 6, is a protein that in humans is encoded by the SLC25A6 gene. Identical copies of this gene reside on the pseudoautosomal regions of the X and Y chromosomes.
SLC25A6 is an ADP/ATP antiporter that facilitates the import of ADP into the mitochondrial matrix for ATP synthesis and the export of ATP out to power the cell. It operates via an alternating access mechanism, switching between a cytoplasmic-open state and a matrix-open state, with a single substrate-binding site exposed to either the cytosol or the mitochondrial matrix. Beyond its primary ADP/ATP transport role, SLC25A6 is implicated in mitochondrial uncoupling and the regulation of the mitochondrial permeability transition pore (mPTP). As a proton transporter, SLC25A6 can uncouple proton flows through the electron transport chain and ATP synthase, decreasing ATP production efficiency and promoting mitochondrial thermogenesis. This proton transport activity is inhibited by the ADP/ATP antiporter function, suggesting that SLC25A6 acts as a crucial regulator of mitochondrial energy output, balancing ATP production and thermogenesis. Free fatty acids are required as cofactors for proton transport but are not transported themselves. SLC25A6 also plays a key role in the opening of mPTP, a non-specific pore that allows the passage of solutes up to 1.5 kDa across mitochondrial membranes, contributing to cell death. However, whether SLC25A6 is a direct pore-forming component or simply regulates mPTP opening remains unclear.
SLC25A6 is also known as AAC3, ANT, ANT 2, ANT 3, ANT3, ANT3Y.
Associated Diseases
- multiple sclerosis
- Parkinson disease
- Alzheimer disease
- lysosomal storage disease
- cancer
- glaucoma
- acute kidney failure
- Timothy syndrome