Solute carrier family 25 member 36 (SLC25A36)

Solute carrier family 25 member 36 (SLC25A36) is a mitochondrial inner membrane transporter responsible for the import and export of pyrimidine nucleotides (such as cytosine and uracil mono-, di-, and tri-phosphates) and guanine nucleotides, utilizing both uniport and antiport mechanisms[1][2][3][5]. This transporter plays a central role in maintaining mitochondrial nucleotide pools, which are essential for mitochondrial DNA and RNA synthesis and degradation, thus supporting mitochondrial genome maintenance and mitochondrial biogenesis[1][2][3][5]. Genetic defects in SLC25A36 have been shown to cause familial hyperinsulinism/hyperammonemia syndrome due to perturbation of mitochondrial nucleotide availability and metabolic imbalance[1][5]. SLC25A36 is not currently a direct drug target, nor are any drugs or established biomarkers linked to it, but loss of function can lead to metabolic and neurological symptoms[1][5].

Other names

PNC2FLJ10618HHF8Solute carrier family 25 (pyrimidine nucleotide carrier), member 36Epididymis secretory sperm binding proteinSLC25A36