MitoProteome is an object-relational mitochondrial gene/protein sequence database and annotation system. The initial release (2004) contained 847 human mitochondrial protein sequences, derived from public sequence databases and mass spectrometric analysis of highly purified human heart mitochondria. The human MitoProteome database was substantially revised in 2015. The original protein-centric system was replaced with a gene-centric infrastructure. Mitochondria-related genes (based on curation of NCBI Entrez gene records, Gene Ontology categories and UniProt annotations) were first obtained, then all corresponding mRNA records for isoforms and splice-variants of each gene were collected, along with the protein sequences themselves. Additionally, all annotations regarding post-translational processing of the gene-product of interest (e.g. removal of peptide sequences going from an apo-protein to mature protein or removal of a targeting sequence from a mitochondrial protein) were also collected. This approach enables an overview of a mitochondria-related gene of interest where all isoforms (and protein sequences) are listed together (including splice variants in the 5' and 3' untranslated regions), along with the protein sequences and annotations of post-translational processing. In addition, a new section was added covering orthologs and homologs of the related proteins with links to data and multiple alignments of NCBI's "Homogene" resource and to hierarchical ortholog information at OrthoDB. The data collection and processing was carried out using an in-house software suite based on BioPython which enables high-throughput programmatic access to key data sources such as NCBI-Entrez and Uniprot. The current version of Mitoproteome contains data for 1,705 genes and 3,625 proteins and is located at the University of California San Diego.

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Search the MitoProteome Database

MITO_ID Entrez Gene_ID Gene Name Gene Symbol Synonyms Alternate names Sub-mitochondrial Compartment Inner membrane Matrix Outer membrane Intermembrane space Other/unspecified Kegg pathway/function 2-Oxocarboxylic acid metabolism ABC transporters Acute myeloid leukemia Acylglycerol degradation Adenine ribonucleotide biosynthesis, IMP => ADP,ATP Adherens junction Adipocytokine signaling pathway Adrenergic signaling in cardiomyocytes African trypanosomiasis Alanine, aspartate and glutamate metabolism Alcoholism Aldosterone-regulated sodium reabsorption Allograft rejection alpha-Linolenic acid metabolism Alzheimer's disease Aminoacyl-tRNA biosynthesis Aminoacyl-tRNA biosynthesis, eukaryotes Amino sugar and nucleotide sugar metabolism Amoebiasis Amphetamine addiction AMPK signaling pathway Amyotrophic lateral sclerosis (ALS) Antigen processing and presentation Apoptosis Arachidonic acid metabolism Arginine and proline metabolism Arrhythmogenic right ventricular cardiomyopathy (ARVC) Ascorbate and aldarate metabolism Autoimmune thyroid disease Axon guidance Bacterial invasion of epithelial cells Basal cell carcinoma Basal transcription factors Base excision repair B cell receptor signaling pathway BER complex beta-Alanine metabolism beta-Oxidation beta-Oxidation, acyl-CoA synthesis Bile acid biosynthesis, cholesterol => cholate/chenodeoxycholate Bile secretion Biosynthesis of amino acids Biosynthesis of unsaturated fatty acids Biotin metabolism Bladder cancer Butanoate metabolism Butirosin and neomycin biosynthesis C19/C18-Steroid hormone biosynthesis, pregnenolone => androstenedione => estrone C1-unit interconversion, eukaryotes C21-Steroid hormone biosynthesis, progesterone => corticosterone/aldosterone C21-Steroid hormone biosynthesis, progesterone => cortisol/cortisone C5 isoprenoid biosynthesis, mevalonate pathway Calcium signaling pathway cAMP signaling pathway Cap binding complex Carbohydrate digestion and absorption Carbon metabolism Cardiac muscle contraction Catecholamine biosynthesis, tyrosine => dopamine => noradrenaline => adrenaline Cell adhesion molecules (CAMs) Cell cycle Ceramide biosynthesis cGMP-PKG signaling pathway Chagas disease (American trypanosomiasis) Chemical carcinogenesis Chemokine signaling pathway Cholecalciferol biosynthesis Cholinergic synapse Chondroitin sulfate degradation Chronic myeloid leukemia Circadian entrainment Circadian rhythm Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate Citrate cycle (TCA cycle) Citrate cycle (TCA cycle, Krebs cycle) Cocaine addiction Coenzyme A biosynthesis, pantothenate => CoA Collecting duct acid secretion Colorectal cancer Creatine pathway Cyanoamino acid metabolism Cysteine and methionine metabolism Cytochrome bc1 complex Cytochrome bc1 complex respiratory unit Cytochrome c oxidase Cytokine-cytokine receptor interaction Cytosolic DNA-sensing pathway D-Arginine and D-ornithine metabolism Degradation of aromatic compounds Dermatan sulfate degradation D-Glutamine and D-glutamate metabolism Dilated cardiomyopathy DNA polymerase delta complex DNA polymerase gamma complex DNA replication Dopaminergic synapse Dorso-ventral axis formation Drug metabolism - cytochrome P450 Drug metabolism - other enzymes ECV complex Endocrine and other factor-regulated calcium reabsorption Endocytosis Endometrial cancer Epithelial cell signaling in Helicobacter pylori infection Epstein-Barr virus infection ErbB signaling pathway ESCRT-II complex ESCRT-III complex Estrogen signaling pathway Ether lipid metabolism FA core complex Fanconi anemia pathway Fat digestion and absorption Fatty acid biosynthesis Fatty acid biosynthesis, elongation Fatty acid biosynthesis, elongation, mitochondria Fatty acid biosynthesis, initiation Fatty acid degradation Fatty acid elongation Fatty acid metabolism Fc epsilon RI signaling pathway Fc gamma R-mediated phagocytosis Focal adhesion Folate biosynthesis FoxO signaling pathway Fructose and mannose metabolism F-type ATPase, eukaryotes GABA biosynthesis, eukaryotes, putrescine => GABA GABAergic synapse GABA (gamma-Aminobutyrate) shunt Galactose metabolism Gap junction Gastric acid secretion Glioma Gluconeogenesis, oxaloacetate => fructose-6P Glucuronate pathway (uronate pathway) Glutamatergic synapse Glutathione biosynthesis, glutamate => glutathione Glutathione metabolism Glycerolipid metabolism Glycerophospholipid metabolism Glycine, serine and threonine metabolism Glycolysis, core module involving three-carbon compounds Glycolysis (Embden-Meyerhof pathway), glucose => pyruvate Glycolysis / Gluconeogenesis Glycosaminoglycan biosynthesis, chondroitin sulfate backbone Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate Glycosaminoglycan degradation Glyoxylate and dicarboxylate metabolism GnRH signaling pathway Graft-versus-host disease Guanine ribonucleotide biosynthesis IMP => GDP,GTP Hedgehog signaling pathway Hematopoietic cell lineage Hepatitis B Hepatitis C Herpes simplex infection HIF-1 signaling pathway Hippo signaling pathway Histidine metabolism Holo-TFIIH complex Homologous recombination HTLV-I infection Huntington's disease Hypertrophic cardiomyopathy (HCM) Immunoproteasome Inflammatory bowel disease (IBD) Inflammatory mediator regulation of TRP channels Influenza A Inosine monophosphate biosynthesis, PRPP + glutamine => IMP Inositol phosphate metabolism Inositol phosphate metabolism, PI=> PIP2 => Ins(1,4,5)P3 => Ins(1,3,4,5)P4 Insulin secretion Insulin signaling pathway Jak-STAT signaling pathway Legionellosis Leishmaniasis Leucine degradation, leucine => acetoacetate + acetyl-CoA Leukocyte transendothelial migration Linoleic acid metabolism Lipoic acid metabolism Long-term depression Long-term potentiation Lysine biosynthesis Lysine degradation Lysine degradation, lysine => saccharopine => acetoacetyl-CoA Lysosome Malaria Malonate semialdehyde pathway, propanoyl-CoA => Acetyl-CoA MAPK signaling pathway Maturity onset diabetes of the young Measles Melanogenesis Melanoma Metabolic pathways Metabolism of xenobiotics by cytochrome P450 Methionine degradation Methionine salvage pathway MicroRNAs in cancer Mineral absorption Mismatch repair Morphine addiction mRNA surveillance pathway mTOR signaling pathway NADH dehydrogenase (ubiquinone) 1 alpha subcomplex NADH dehydrogenase (ubiquinone) 1 beta subcomplex NADH dehydrogenase (ubiquinone) Fe-S protein/flavoprotein complex, mitochondria Natural killer cell mediated cytotoxicity Neuroactive ligand-receptor interaction Neurotrophin signaling pathway NF-kappa B signaling pathway N-Glycan biosynthesis N-glycan biosynthesis, complex type N-glycosylation by oligosaccharyltransferase Nicotinate and nicotinamide metabolism Nitrogen metabolism NOD-like receptor signaling pathway Non-alcoholic fatty liver disease (NAFLD) Non-homologous end-joining Non-small cell lung cancer Notch signaling pathway Nucleotide excision repair Nucleotide sugar biosynthesis, eukaryotes Nucleotide sugar biosynthesis, glucose => UDP-glucose Olfactory transduction One carbon pool by folate Oocyte meiosis Osteoclast differentiation Other glycan degradation Other types of O-glycan biosynthesis Ovarian steroidogenesis Oxidative phosphorylation Oxytocin signaling pathway p53 signaling pathway Pancreatic cancer Pancreatic secretion Pantothenate and CoA biosynthesis Parkinson's disease Pathogenic Escherichia coli infection Pathways in cancer Pentose and glucuronate interconversions Pentose phosphate pathway Pentose phosphate pathway, oxidative phase, glucose 6P => ribulose 5P Pentose phosphate pathway (Pentose phosphate cycle) Peroxisome Pertussis Phagosome Phenylalanine metabolism Phenylalanine, tyrosine and tryptophan biosynthesis Phosphatidylcholine (PC) biosynthesis, PE => PC Phosphatidylinositol signaling system Phototransduction PI3K-Akt signaling pathway Platelet activation Polyamine biosynthesis, arginine => agmatine => putrescine => spermidine Polyamine biosynthesis, arginine => ornithine => putrescine Porphyrin and chlorophyll metabolism PPAR signaling pathway Primary bile acid biosynthesis Primary immunodeficiency Prion diseases Progesterone-mediated oocyte maturation Prolactin signaling pathway Proline biosynthesis, glutamate => proline Propanoate metabolism Prostate cancer Proteasome Proteasome, 20S core particle Protein digestion and absorption Protein export Protein processing in endoplasmic reticulum Proteoglycans in cancer Proximal tubule bicarbonate reclamation Purine metabolism Pyrimidine metabolism Pyrimidine ribonucleotide biosynthesis, UMP => UDP/UTP,CDP/CTP Pyruvate metabolism Pyruvate oxidation, pyruvate => acetyl-CoA Rap1 signaling pathway Ras signaling pathway Regulation of actin cytoskeleton Regulation of autophagy Renal cell carcinoma Renin-angiotensin system Retinol metabolism Retrograde endocannabinoid signaling Rheumatoid arthritis Riboflavin metabolism Ribosome Ribosome biogenesis in eukaryotes Ribosome, eukaryotes RIG-I-like receptor signaling pathway RNA degradation RNA transport Salivary secretion Salmonella infection Selenocompound metabolism Serotonergic synapse Shigellosis Signaling pathways regulating pluripotency of stem cells Small cell lung cancer SNARE interactions in vesicular transport Sphingolipid metabolism Sphingosine biosynthesis Spliceosome Spliceosome, 35S U5-snRNP Spliceosome, Prp19/CDC5L complex Spliceosome, U4/U6.U5 tri-snRNP Starch and sucrose metabolism Steroid biosynthesis Steroid hormone biosynthesis Steroid hormone biosynthesis, cholesterol => prognenolone => progesterone Succinate dehydrogenase (ubiquinone) Sulfur metabolism Sulfur relay system Synaptic vesicle cycle Synthesis and degradation of ketone bodies Taste transduction Taurine and hypotaurine metabolism T cell receptor signaling pathway Terpenoid backbone biosynthesis TGF-beta signaling pathway Thiamine metabolism Thyroid cancer Thyroid hormone biosynthesis, tyrosine => triiodothyronine/thyroxine Thyroid hormone signaling pathway Thyroid hormone synthesis Tight junction TNF signaling pathway Toll-like receptor signaling pathway Toxoplasmosis Transcriptional misregulation in cancer Triacylglycerol biosynthesis Tryptophan metabolism Tuberculosis Type I diabetes mellitus Type II diabetes mellitus Tyrosine degradation, tyrosine => homogentisate Tyrosine metabolism Ubiquinone and other terpenoid-quinone biosynthesis Ubiquinone biosynthesis, eukaryotes, 4-hydroxybenzoate => ubiquinone Ubiquitin mediated proteolysis Urea cycle Uridine monophosphate biosynthesis, glutamine (+ PRPP) => UMP Valine, leucine and isoleucine biosynthesis Valine, leucine and isoleucine degradation Vascular smooth muscle contraction Vasopressin-regulated water reabsorption VEGF signaling pathway Vibrio cholerae infection Viral carcinogenesis Viral myocarditis Vitamin digestion and absorption V-type ATPase, eukaryotes Wnt signaling pathway Records per page 10 20 50 100 200 500 1000 All Sort by: Gene Symbol Species Entrez Gene ID Gene Name MITO_ID

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Download MitoProteome (1/18/2022)

Download MitoProteome as a set of Postgres database tables: Download zip file (3.1MB)
Contents: mt_main, mt_gene, mt_protein, mt_taxon, mt_domains, mt_enzyme, mt_ontology, mt_orthologs, mt_pathways, mt_similar_protein, mt_comments.

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Literature References

• MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins (2016)
  Calvo, S.E., Klauser, C.R., Mootha, V.K. Nucl. Acids Res. 44 (D1): D1251-D1257. doi: 10.1093/nar/gkv1003
• A mitochondrial protein compendium elucidates complex I disease biology. (2008)
  Pagliarini, D.J., Calvo, S.E., Chang, B., Sheth, S.A., Vafai, S.B., Ong, S.E., Walford, G.A., Sugiana, C., Boneh, A., Chen, W.K., et al. Cell 134, 112-123 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2778844/
• MitoProteome: Mitochondrial Protein Sequence Database and Annotation System. (2004)
  Dawn Cotter, Purnima Guda, Eoin Fahy, Shankar Sumbramaniam. Nucleic Acids Research 2004 32: D463-D467.
• Characterization of the human heart mitochondrial proteome (2003)
  Steven W. Taylor, Eoin Fahy, Bing Zhang, Gary M. Glenn, Dale E. Warnock, Sandra Wiley, Anne N. Murphy, Sara P. Gaucher, Roderick A. Capaldi, Bradford W. Gibson, and Soumitra S. Ghosh. Nature Biotechnology March 2003 Volume 21 Number 3 pp 281 - 286.