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ADP/ATP translocase, adenine nucleotide translocase (ANT) or ADP/ATP carrier protein
Function:
- voltage-gated channel of inner mitochondrial membrane
- in association with VDAC-1 (outer mitochondrial membrane), ANT is thought to form the major pathway for movement of adenine nucleotides (ATP, ADP) through the outer mitochondrial membrane, the mitochondrial permeability transition pore
- opening of ANT dissipates the H+ gradient across the inner mitochondrial membrane, uncoupling oxidative phosphorylation
- may serve as the mitochondrial binding site for cyclophilin D
Pathology:
- mutations in the gene for ANT-1 are associated with large deletions in mitochondrial DNA, possibly due to deficient adenine nucleotide pools for mitochondrial DNA synthesis or lack of a sufficient energy source for synthesis of mitochondrial DNA, or maintenance of its integrity
Specific
ADP/ATP translocase-1 (adenine nucleotide translocator 1, ANT 1, ADP-ATP carrier protein 1, solute carrier family 25 member 4, heart/skeletal ADP/ATP carrier protein, SLC25A4, ANT1)
ADP/ATP translocase-2 (adenine nucleotide translocator 2, ANT 2, ADP-ATP carrier protein 2, solute carrier family 25 member 5, fibroblast ADP/ATP carrier protein)
ADP/ATP translocase-3 (solute carrier family 25 member 6, SLC25A6, liver ADP/ATP carrier protein)
ADP/ATP translocase-3 (Y chromosome)
General
carrier protein (transporter)
mitochondrial membrane protein
oligomerizing protein
voltage-gated ion channel
Properties
CONFIGURATION: dimer
SIZE: MW = 30 kD
COMPARTMENT: inner mitochondrial membrane
MOTIF: membrane region
ion channel
COMPARTMENT: cellular membrane
MOTIF: membrane region
STATE: M-STATE
MISC-INFO: INHIBITOR = ATRACTYLOSIDE
INHIBITOR = BONGKREKATE
Database Correlations
OMIM 103220
MORBIDMAP 103220
References
- Rottenberg H, Marbach M.
Regulation of Ca2+ transport in brain mitochondria. I.
The mechanism of spermine enhancement of Ca2+ uptake and
retention.
Biochim Biophys Acta. 1990 Mar 15;1016(1):77-86.
PMID: 2310743
- Rottenberg H, Marbach M.
Regulation of Ca2+ transport in brain mitochondria. II.
The mechanism of the adenine nucleotides enhancement of Ca2+
uptake and retention.
Biochim Biophys Acta. 1990 Mar 15;1016(1):87-98.
PMID: 2310744
- OMIM :accession 103220
- Moraes CT.
A helicase is born.
Nat Genet. 2001 Jul;28(3):200-1.
PMID: 11431681
- Spelbrink JN, Li FY, Tiranti V, Nikali K, Yuan QP, et al
Human mitochondrial DNA deletions associated with mutations
in the gene encoding Twinkle, a phage T7 gene 4-like protein
localized in mitochondria.
Nat Genet. 2001 Jul;28(3):223-31.
Erratum in: Nat Genet 2001 Sep;29(1):100.
PMID: 11431692
- Adams JM, Cory S.
Life-or-death decisions by the Bcl-2 protein family.
Trends Biochem Sci. 2001 Jan;26(1):61-6. Review.
PMID: 11165519