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SLC25A1 | Tricarboxylate transport protein, mitochondrial; Involved in citrate-H(+)/malate exchange. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD(+) for the glycolytic pathway; Solute carriers (311 aa) | |||
ACO2 | Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family (780 aa) | |||
ODC1 | Ornithine decarboxylase; Catalyzes the first and rate-limiting step of polyamine biosynthesis that converts ornithine into putrescine, which is the precursor for the polyamines, spermidine and spermine. Polyamines are essential for cell proliferation and are implicated in cellular processes, ranging from DNA replication to apoptosis; Belongs to the Orn/Lys/Arg decarboxylase class-II family (461 aa) | |||
SSR1 | Translocon-associated protein subunit alpha; TRAP proteins are part of a complex whose function is to bind calcium to the ER membrane and thereby regulate the retention of ER resident proteins. May be involved in the recycling of the translocation apparatus after completion of the translocation process or may function as a membrane-bound chaperone facilitating folding of translocated proteins; Minor histocompatibility antigens (286 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) | |||
PCSK4 | Proprotein convertase subtilisin/kexin type 4; Proprotein convertase involved in the processing of hormone and other protein precursors at sites comprised of pairs of basic amino acid residues (By similarity). In males, important for ADAM2 processing as well as other acrosomal proteins with roles in fertilization and critical for normal fertilization events such as sperm capacitation, acrosome reaction and binding of sperm to zona pellucida (By similarity). Plays also a role in female fertility, involved in the regulation of trophoblast migration and placental development, may be throu [...] (755 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) | |||
OAZ3 | Ornithine decarboxylase antizyme 3; Ornithine decarboxylase (ODC) antizyme protein that negatively regulates ODC activity and intracellular polyamine biosynthesis and uptake in response to increased intracellular polyamine levels. Binds to ODC monomers, inhibiting the assembly of the functional ODC homodimers. Does not target the ODC monomers for degradation, which allows a protein synthesis-independent restoration of ODC activity. Stabilizes AZIN2 by interfering with its ubiquitination. Involved in the translocation of AZNI2 from ER-Golgi intermediate compartment (ERGIC) to the cytoso [...] (190 aa) | |||
PXMP2 | Peroxisomal membrane protein 2; Seems to be involved in pore-forming activity and may contribute to the unspecific permeability of the peroxisomal membrane (195 aa) | |||
MINOS1 | MICOS complex subunit MIC10; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane (78 aa) | |||
SLC25A20 | Mitochondrial carnitine/acylcarnitine carrier protein; Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway; Solute carriers (301 aa) | |||
FRAS1 | Fraser extracellular matrix complex subunit 1 (1976 aa) | |||
SLC25A21 | Mitochondrial 2-oxodicarboxylate carrier; Transports C5-C7 oxodicarboxylates across the inner membranes of mitochondria. Can transport 2-oxoadipate, 2- oxoglutarate, adipate, glutarate, and to a lesser extent, pimelate, 2-oxopimelate, 2-aminoadipate, oxaloacetate, and citrate; Belongs to the mitochondrial carrier (TC 2.A.29) family (299 aa) | |||
PPIE | Peptidyl-prolyl cis-trans isomerase E; PPIases accelerate the folding of proteins. It catalyzes the cis-trans isomerization of proline imidic peptide bonds in oligopeptides. Combines RNA-binding and PPIase activities. May be involved in muscle- and brain-specific processes. May be involved in pre-mRNA splicing; Cyclophilin peptidylprolyl isomerases (314 aa) | |||
MPV17 | Protein Mpv17; Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance; Belongs to the peroxisomal membrane protein PXMP2/4 family (176 aa) | |||
MPV17L | Mpv17-like protein; Isoform 1 participates in reactive oxygen species metablism by up- or down-regulation of the genes of antioxidant enzymes; Belongs to the peroxisomal membrane protein PXMP2/4 family (196 aa) | |||
SLC25A45 | Solute carrier family 25 member 45; Belongs to the mitochondrial carrier (TC 2.A.29) family (288 aa) | |||
TBX20 | T-box transcription factor TBX20; Acts as a transcriptional activator and repressor required for cardiac development and may have key roles in the maintenance of functional and structural phenotypes in adult heart; T-boxes (447 aa) | |||
SLC25A48 | Solute carrier family 25 member 48 (157 aa) | |||
ENSG00000261130 | Uncharacterized protein; Belongs to the peroxisomal membrane protein PXMP2/4 family (118 aa) | |||
DNASE1L2 | Deoxyribonuclease-1-like 2; Divalent cation-dependent acid DNA endonuclease involved in the breakdown of the nucleus during corneocyte formation of epidermal keratinocytes. May play an immune role by eliminating harmful DNA released into the extracellular environment by damaged epidermal cells (299 aa) | |||
ATPAF1 | ATP synthase mitochondrial F1 complex assembly factor 1; May play an essential role for the assembly of the mitochondrial F1-F0 complex (351 aa) | |||
MPV17L2 | Mpv17-like protein 2; Required for the assembly and stability of the mitochondrial ribosome. Is a positive regulator of mitochondrial protein synthesis; Belongs to the peroxisomal membrane protein PXMP2/4 family (206 aa) | |||
TAZ | Tafazzin; Some isoforms may be involved in cardiolipin (CL) metabolism; Belongs to the taffazin family (292 aa) | |||
NOSIP | Nitric oxide synthase-interacting protein; E3 ubiquitin-protein ligase that is essential for proper development of the forebrain, the eye, and the face. Catalyzes monoubiquitination of serine/threonine-protein phosphatase 2A (PP2A) catalytic subunit PPP2CA/PPP2CB (By similarity). Negatively regulates nitric oxide production by inducing NOS1 and NOS3 translocation to actin cytoskeleton and inhibiting their enzymatic activity; Belongs to the NOSIP family (301 aa) | |||
OAZ1 | Ornithine decarboxylase antizyme 1; Ornithine decarboxylase (ODC) antizyme protein that negatively regulates ODC activity and intracellular polyamine biosynthesis and uptake in response to increased intracellular polyamine levels. Binds to ODC monomers, inhibiting the assembly of the functional ODC homodimer, and targets the monomers for ubiquitin-independent proteolytic destruction by the 26S proteasome. Triggers ODC degradation by inducing the exposure of a cryptic proteasome-interacting surface of ODC. Stabilizes AZIN2 by interfering with its ubiquitination. Also inhibits cellular u [...] (228 aa) |