ATP-binding cassette sub-family B member 10, mitochondrial; May mediate critical mitochondrial transport functions related to heme biosynthesis; Belongs to the ABC transporter superfamily. ABCB family. Mitochondrial peptide exporter (TC 3.A.1.212) subfamily.

Frataxin intermediate form; Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization. Modulates the RNA-binding activity of ACO1 (By similarity); Belongs to the frataxin family.

Ferrochelatase, mitochondrial; Catalyzes the ferrous insertion into protoporphyrin IX.

ATP-binding cassette sub-family B member 7, mitochondrial; Could be involved in the transport of heme from the mitochondria to the cytosol. Plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins.

Ferritin, mitochondrial; Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Has ferroxidase activity. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation (By similarity); Belongs to the ferritin family.

tRNA (guanine-N(7)-)-methyltransferase; Methyltransferase that mediates the formation of N(7)- methylguanine in a subset of RNA species, such as tRNAs, mRNAs and microRNAs (miRNAs). Catalyzes the formation of N(7)- methylguanine at position 46 (m7G46) in tRNA. Also acts as a methyltransferase for a subset of internal N(7)-methylguanine in mRNAs. Internal N(7)-methylguanine methylation of mRNAs regulates translation. Also methylates a specific subset of miRNAs, such as let-7. N(7)-methylguanine methylation of let- 7 miRNA promotes let-7 miRNA processing by disrupting an inhibitory secon [...]

Natural resistance-associated macrophage protein 2; May serve to import iron into the mitochondria (By similarity). Important in metal transport, in particular iron. Involved in apical iron uptake into duodenal enterocytes. Involved in iron transport from acidified endosomes into the cytoplasm of erythroid precursor cells. May play an important role in hepatic iron accumulation and tissue iron distribution.

Cysteine desulfurase, mitochondrial; Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe- S) clusters. May be involved in the biosynthesis of molybdenum cofactor (By similarity); Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. NifS/IscS subfamily.

Mitochondrial potassium channel ATP-binding subunit; ATP-binding subunit of the mitochondrial potassium channel located in the mitochondrial inner membrane (By similarity). Together with CCDC51/MITOK, forms a protein complex localized in the mitochondria that mediates ATP-dependent potassium currents across the inner membrane (that is, mitoK(ATP) channel) (By similarity). Plays a role in mitochondrial iron transport. Required for maintenance of normal cardiac function, possibly by influencing mitochondrial iron export and regulating the maturation of cytosolic iron sulfur cluster-conta [...]