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