Reactome | AAC1 imports a proton

AAC1 imports a proton

Stable Identifier

R-HSA-9864415

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial intermembrane space, mitochondrial matrix

ReviewStatus

5/5

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ADP/ATP translocase 1 (ANT1, AAC1, SLC25A4) transports a proton from the mitochondrial intermembrane space to the mitochondrial matrix, generating heat. The reaction does not occur without activation by fatty acids binding to the transporter (Bertholet et al., 2019; Kreiter et al., 2023; reviewed in Bertholet & Kirichok, 2022). Defects in AAC1 can lead to diseases affecting ocular or heart muscle cells (reviewed in Mishra et al., 2023).

Literature References

PubMed ID	Title	Journal	Year
31341297	H⁺ transport is an integral function of the mitochondrial ADP/ATP carrier Terada, N, Spiegelman, BM, Kirichok, Y, Cho, J, Chouchani, ET, Vidoni, S, Angelin, A, Garrity, R, Wallace, DC, Long, JZ, Kazak, L, Fedorenko, A, Bertholet, AM	Nature	2019
37762012	FA Sliding as the Mechanism for the ANT1-Mediated Fatty Acid Anion Transport in Lipid Bilayers Kreiter, J, Škulj, S, Pohl, EE, Vazdar, M, Brkljača, Z, Bardakji, S	Int J Mol Sci	2023
34758268	Mitochondrial H⁺ Leak and Thermogenesis Kirichok, Y, Bertholet, AM	Annu Rev Physiol	2022
37449547	Adenine nucleotide carrier protein dysfunction in human disease Coyne, LP, Chen, XJ, Mishra, G	IUBMB Life	2023