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SLC25A4 Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. Defects in SLC25A4 are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 2 (PEOA2). Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged- red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism. Belongs to the mitochondrial carrier family. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Transporter; Transporter, SLC family
Chromosomal Location of Human Ortholog: 4q35.1
Cellular Component: integral component of plasma membrane; mitochondrial inner membrane; mitochondrion; myelin sheath; nucleus
Molecular Function: adenine transmembrane transporter activity; ATP:ADP antiporter activity; protein binding
Biological Process: adenine transport; ADP transport; apoptotic mitochondrial changes; ATP transport; generation of precursor metabolites and energy; mitochondrial genome maintenance; regulation of insulin secretion; regulation of mitochondrial membrane permeability; transmembrane transport; transport; viral process
Disease: Mitochondrial Dna Depletion Syndrome 12 (cardiomyopathic Type); Mitochondrial Dna Depletion Syndrome 12a (cardiomyopathic Type), Autosomal Dominant; Progressive External Ophthalmoplegia With Mitochondrial Dna Deletions, Autosomal Dominant, 2
Reference #:  P12235 (UniProtKB)
Alt. Names/Synonyms: AAC1; Adenine nucleotide translocator 1; adenine nucleotide translocator 1 (skeletal muscle); adenine nucleotide translocator), member 4; ADP,ATP carrier protein 1; ADP,ATP carrier protein, heart/skeletal muscle; ADP,ATP carrier protein, heart/skeletal muscle isoform T1; ADP/ATP translocase 1; ADT1; ANT; ANT 1; ANT1; heart/skeletal muscle ATP/ADP translocator; PEO2; PEO3; SLC25A4; solute carrier family 25 (mitochondrial carrier; Solute carrier family 25 member 4; T1
Gene Symbols: SLC25A4
Molecular weight: 33,064 Da
Basal Isoelectric point: 9.78  Predict pI for various phosphorylation states
CST Pathways:  Warburg Effect
Select Structure to View Below


Protein Structure Not Found.
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