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ACO2 | Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family (780 aa) | |||
ALLC | Probable allantoicase; The function of this enzyme is unclear as allantoicase activity is not known to exist in mammals (391 aa) | |||
ABCB7 | 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; ATP binding cassette subfamily B (753 aa) | |||
IREB2 | Iron-responsive element-binding protein 2; RNA-binding protein that binds to iron-responsive elements (IRES), which are stem-loop structures found in the 5’- UTR of ferritin, and delta aminolevulinic acid synthase mRNAs, and in the 3’-UTR of transferrin receptor mRNA. Binding to the IRE element in ferritin results in the repression of its mRNA translation. Binding of the protein to the transferrin receptor mRNA inhibits the degradation of this otherwise rapidly degraded mRNA; Belongs to the aconitase/IPM isomerase family (963 aa) | |||
NUBP2 | Cytosolic Fe-S cluster assembly factor NUBP2; Component of the cytosolic iron-sulfur (Fe/S) protein assembly (CIA) machinery. Required for maturation of extramitochondrial Fe-S proteins. The NUBP1-NUBP2 heterotetramer forms a Fe-S scaffold complex, mediating the de novo assembly of an Fe-S cluster and its transfer to target apoproteins. Negatively regulates cilium formation and structure (271 aa) | |||
ABCB6 | ATP-binding cassette sub-family B member 6, mitochondrial; Binds heme and porphyrins and functions in their ATP- dependent uptake into the mitochondria. Plays a crucial role in heme synthesis; Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily (842 aa) | |||
SLC2A12 | Solute carrier family 2, facilitated glucose transporter member 12; Facilitative glucose transporter; Belongs to the major facilitator superfamily. Sugar transporter (TC 2.A.1.1) family. Glucose transporter subfamily (617 aa) | |||
NUBPL | Iron-sulfur protein NUBPL; Required for the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). May deliver of one or more Fe-S clusters to complex I subunits; Belongs to the Mrp/NBP35 ATP-binding proteins family (319 aa) | |||
SLC25A31 | ADP/ATP translocase 4; Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. May serve to mediate energy generating and energy consuming processes in the distal flagellum, possibly as a nucleotide shuttle between flagellar glycolysis, protein phosphorylation and mechanisms of motility; Belongs to the mitochondrial carrier (TC 2.A.29) family (315 aa) | |||
SLC25A4 | ADP/ATP translocase 1; Involved in mitochondrial ADP/ATP transport. Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane; Belongs to the mitochondrial carrier (TC 2.A.29) family (298 aa) | |||
NUBP1 | Cytosolic Fe-S cluster assembly factor NUBP1; Component of the cytosolic iron-sulfur (Fe/S) protein assembly (CIA) machinery. Required for maturation of extramitochondrial Fe-S proteins. The NUBP1-NUBP2 heterotetramer forms a Fe-S scaffold complex, mediating the de novo assembly of an Fe-S cluster and its transfer to target apoproteins. Implicated in the regulation of centrosome duplication (By similarity). Negatively regulates cilium formation and structure (By similarity) (320 aa) | |||
LETM1 | Mitochondrial proton/calcium exchanger protein; Mitochondrial proton/calcium antiporter that mediates proton-dependent calcium efflux from mitochondrion. Crucial for the maintenance of mitochondrial tubular networks and for the assembly of the supercomplexes of the respiratory chain. Required for the maintenance of the tubular shape and cristae organization. In contrast to SLC8B1/NCLX, does not constitute the major factor for mitochondrial calcium extrusion; Belongs to the LETM1 family (739 aa) | |||
SLC25A33 | Solute carrier family 25 member 33; Mitochondrial transporter that imports/exports pyrimidine nucleotides into and from mitochondria. Transports preferentially uracil, thymine, and cytosine (deoxy)nucleoside di- and triphosphates by an antiport mechanism. Also transports guanine but not adenine (deoxy)nucleotides. Is inhibited strongly by pyridoxal 5’-phosphate, 4,7-diphenyl-1,10- phenanthroline, tannic acid, and mercurials (mercury dichloride, mersalyl acid, p-hydroxymercuribenzoate). Participates in mitochondrial genome maintenance, regulation of mitochondrial membrane potential and [...] (321 aa) | |||
ACO1 | Cytoplasmic aconitate hydratase; Iron sensor. Binds a 4Fe-4S cluster and functions as aconitase when cellular iron levels are high. Functions as mRNA binding protein that regulates uptake, sequestration and utilization of iron when cellular iron levels are low. Binds to iron-responsive elements (IRES) in target mRNA species when iron levels are low. Binding of a 4Fe-4S cluster precludes RNA binding (889 aa) | |||
SLC25A36 | Solute carrier family 25 member 36; Mitochondrial transporter that imports/exports pyrimidine nucleotides into and from mitochondria. Transports preferentially cytosine and uracil (deoxy)nucleoside mono-, di-, and triphosphates by uniport and antiport mechanism. Also transports guanine but not adenine (deoxy)nucleotides. Is inhibited strongly by pyridoxal 5’-phosphate, 4,7-diphenyl-1,10- phenanthroline, tannic acid, and mercurials (mercury dichloride, Mersalyl acid, p-hydroxymercuribenzoate). Participates in mitochondrial genome maintenance, regulation of mitochondrial membrane potenti [...] (311 aa) | |||
PDCD1 | Programmed cell death protein 1; Inhibitory cell surface receptor involved in the regulation of T-cell function during immunity and tolerance. Upon ligand binding, inhibits T-cell effector functions in an antigen- specific manner. Possible cell death inducer, in association with other factors; CD molecules (288 aa) | |||
CS | Citrate synthase, mitochondrial; Citrate synthase (466 aa) | |||
SLC25A28 | Mitoferrin-2; Mitochondrial iron transporter that mediates iron uptake. Probably required for heme synthesis of hemoproteins and Fe-S cluster assembly in non-erythroid cells. The iron delivered into the mitochondria, presumably as Fe(2+), is then probably delivered to ferrochelatase to catalyze Fe(2+) incorporation into protoprophyrin IX to make heme (By similarity); Belongs to the mitochondrial carrier (TC 2.A.29) family (364 aa) | |||
SLC25A5 | ADP/ATP translocase 2; Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. As part of the mitotic spindle-associated MMXD complex it may play a role in chromosome segregation; Solute carriers (298 aa) | |||
SLC15A1 | Solute carrier family 15 member 1; Proton-coupled intake of oligopeptides of 2 to 4 amino acids with a preference for dipeptides. May constitute a major route for the absorption of protein digestion end-products; Solute carriers (708 aa) | |||
FXN | Frataxin, mitochondrial; 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; however, the physiological relevance is unsure as reports are conflicting and the function has only [...] (210 aa) | |||
LETM2 | LETM1 domain-containing protein LETM2, mitochondrial; Leucine zipper and EF-hand containing transmembrane protein 2 (491 aa) | |||
SLC25A6 | ADP/ATP translocase 3; Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. May participate in the formation of the permeability transition pore complex (PTPC) responsible for the release of mitochondrial products that triggers apoptosis; Belongs to the mitochondrial carrier (TC 2.A.29) family (298 aa) | |||
CIAPIN1 | Anamorsin; Has anti-apoptotic effects in the cell. Involved in negative control of cell death upon cytokine withdrawal. Promotes development of hematopoietic cells (By similarity). Component of the cytosolic iron-sulfur (Fe-S) protein assembly (CIA) machinery. Required for the maturation of extramitochondrial Fe-S proteins. Part of an electron transfer chain functioning in an early step of cytosolic Fe-S biogenesis. Electrons are transferred to the Fe-S cluster from NADPH via the FAD- and FMN-containing protein NDOR1 (312 aa) | |||
MRS2 | Magnesium transporter MRS2 homolog, mitochondrial; Magnesium transporter that mediates the influx of magnesium into the mitochondrial matrix. Required for normal expression of the mitochondrial respiratory complex I subunits; Belongs to the CorA metal ion transporter (MIT) (TC 1.A.35) family (446 aa) | |||
ENSG00000144785 | Uncharacterized protein (263 aa) |