The sideroflexin (SFXN) family is comprised of SFXN1, SFXN2, SFXN3, SFXN4 and SFXN5. SFXN1, also designated tricarboxylate carrier protein TCC, is the most highly characterized family member. The ubiquitously expressed SFXN1 protein resides as an integral protein of the mitochondrial inner membrane. It functions as an essential component of the shuttle system that transports mitochondrial acetyl-CoA into the cytosol, where lipogenesis occurs. The SFXN1 gene is mutated in flexed-tail (f/f) mice, which display axial skeletal abnormalities and a transient embryonic and neonatal anemia characterized by pathologic intramitochondiral iron deposits in erythrocytes. Therefore, SFXN1 is also thought to facilitate the transport of a component required for iron utilization into mitochondria. All SFXN family members show expression in pancreatic islet cells. SFXN5 displays a citrate transport activity and is primarily expressed in brain.
Function:
Might be involved in the transport of a component required for iron utilization into or out of the mitochondria.
Subcellular Location:
Mitochondrion membrane.
Similarity:
Belongs to the sideroflexin family.
SWISS:
Q9H9B4
Gene ID:
94081
Database links:
Entrez Gene: 94081 Human
Entrez Gene: 14057 Mouse
Entrez Gene: 364678 Rat
GenBank: NM_022754 Human
SwissProt: Q9H9B4 Human
SwissProt: Q99JR1 Mouse
SwissProt: Q63965 Rat
Unigene: 36988 Human
Unigene: 134191 Mouse
Unigene: 115752 Rat
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