SFXN1

Might be involved in the transport of a component required for iron utilization into or out of the mitochondria.

Full Name

Sideroflexin 1

Function

Amino acid transporter importing serine, an essential substrate of the mitochondrial branch of the one-carbon pathway, into mitochondria. Mitochondrial serine is then converted to glycine and formate, which exits to the cytosol where it is used to generate the charged folates that serve as one-carbon donors (PubMed:30442778).
May also transport other amino acids including alanine and cysteine (PubMed:30442778).

Biological Process

Biological Process erythrocyte differentiationIEA:Ensembl
Biological Process iron ion transportIEA:Ensembl
Biological Process L-alanine transportManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process L-serine transportManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process mitochondrial transmembrane transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process one-carbon metabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process serine import into mitochondrionManual Assertion Based On ExperimentIDA:UniProtKB

Cellular Location

Mitochondrion inner membrane

Topology

Mitochondrial matrix: 2-102
Helical: 103-120
Mitochondrial intermembrane: 121-146
Helical: 147-167
Mitochondrial matrix: 168-174
Helical: 175-195
Mitochondrial intermembrane: 196-228
Helical: 229-249
Mitochondrial matrix: 250-266
Helical: 267-287
Mitochondrial intermembrane: 288-322