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Home >> Signaling Pathways >> Proteases >> Mitochondrial Metabolism

Mitochondrial Metabolism

Mitochondria use multiple carbon fuels to produce ATP and metabolites, including pyruvate, which is generated from glycolysis; amino acids such as glutamine; and fatty acids. These carbon fuels feed into the TCA cycle in the mitochondrial matrix to generate the reducing equivalents NADH and FADH2, which deliver their electrons to the electron transport chain. Mitochondria are complex organelles that play an important role in many facets of cellular function, from metabolism to immune regulation and cell death. Mitochondria are actively involved in a wide variety of cellular processes and molecular interactions, such as calcium buffering, lipid flux, and intracellular signaling. It is increasingly recognized that mitochondrial dysfunction is a hallmark of many diseases such as obesity/diabetes, cancer, cardiovascular and neurodegenerative diseases. Mitochondrial metabolism is a key determinant of tumor progression by impacting on functions such as epithelial-to-mesenchymal transition. Mitochondrial metabolism and derived oncometabolites shape the epigenetic landscape to alter aggressiveness features of cancer cells. Changes in mitochondrial metabolism are relevant for the survival of tumors in response to therapy.

Targets for  Mitochondrial Metabolism

Products for  Mitochondrial Metabolism

  1. Cat.No. Product Name Information
  2. GC62137 IMT1 IMT1 is a first-in-class specific and noncompetitive human mitochondrial RNA polymerase (POLRMT) inhibitor. IMT1 Chemical Structure
  3. GC43909 Iromycin A Iromycin A is a bacterial pyridone metabolite that inhibits nitric oxide synthase (NOS) activity, with selectivity for NOS III (endothelial NOS) over NOS I (neuronal NOS). Iromycin A Chemical Structure
  4. GC43912 Isoapoptolidin Isoapoptolidin is a stable derivative of apoptolidin that demonstrates 10-fold reduced F1FO-ATP synthase inhibitory activity compared to apoptolidin (IC50s = 17 and 0.7 μM, respectively). Isoapoptolidin Chemical Structure
  5. GC43922 Isovaleryl-L-carnitine (chloride) Isovaleryl-L-carnitine (chloride), a product of the catabolism of L-leucine, is a potent activator of the Ca2+-dependent proteinase (calpain) of human neutrophils. Isovaleryl-L-carnitine (chloride) Chemical Structure
  6. GC47531 Kresoxim-methyl A fungicide Kresoxim-methyl Chemical Structure
  7. GC47548 L-Carnitine-d3 (chloride) L-Carnitine-d3 ((R)-Carnitine-d3) hydrochloride is the deuterium labeled L-Carnitine. L-Carnitine-d3 (chloride) Chemical Structure
  8. GC52094 L-Carnitine-d9 (chloride) An internal standard for the quantification of L-carnitine L-Carnitine-d9 (chloride) Chemical Structure
  9. GC49382 L-Lysine-d3 (hydrochloride) An internal standard for the quantification of L-lysine L-Lysine-d3 (hydrochloride) Chemical Structure
  10. GC19221 Leucomethylene blue Mesylate Leucomethylene blue (Mesylate) is a common reduced form of Methylene Blue, Methylene Blue is a member of the thiazine class of dyes. Leucomethylene blue Mesylate Chemical Structure
  11. GC63973 Ligustroside Ligustroside (Ligstroside), a secoiridoid derivative, has outstanding performance on mitochondrial bioenergetics in models of early Alzheimer's disease (AD) and brain ageing by mechanisms that may not interfere with Aβ production. Ligustroside Chemical Structure
  12. GN10423 Lipoic acid Lipoic acid Chemical Structure
  13. GC11532 Lonidamine hexokinase inactivator Lonidamine Chemical Structure
  14. GC48989 LTX-315 (trifluoroacetate salt) A synthetic cationic amphiphilic peptide LTX-315 (trifluoroacetate salt) Chemical Structure
  15. GC44093 Lutein Lutein is a natural yellow carotenoid, which can be found in plants, egg yolks, and in the human retina. Lutein Chemical Structure
  16. GC40007 Malformin A Malformin A is a cyclopentapeptide fungal metabolite that has been found in A. Malformin A Chemical Structure
  17. GC61790 MCU-i4 MCU-i4 blocks the IP3-dependent mitochondrial Ca2+-uptake, maintaining the gatekeeping role of their target. MCU-i4 Chemical Structure
  18. GC66184 Menadiol Menadiol (Dihydrovitamin K3), a menaquinol analogue, is an electron donor for reversed oxidative phosphorylation in submitochondrial particles. Menadiol Chemical Structure
  19. GC47620 Mensacarcin A bacterial metabolite with anticancer activity Mensacarcin Chemical Structure
  20. GC47663 Methylene Blue (hydrate) A cationic dye with diverse biological activities Methylene Blue (hydrate) Chemical Structure
  21. GC49472 Methylmalonic Acid-d3 Methylmalonic acid-d3 (Methylpropanedioic acid-d3) is the deuterium labeled Methylmalonic acid. Methylmalonic acid (Methylmalonate) is an indicator of Vitamin B-12 deficiency in cancer. Methylmalonic Acid-d3 Chemical Structure
  22. GC65989 MFI8 MFI8 is a small molecule inhibitor of mitochondrial fusion. MFI8 Chemical Structure
  23. GC47685 MIT-PZR A mitochondria-targeted fluorescent probe MIT-PZR Chemical Structure
  24. GC62564 Mito-LND Mito-LND (Mito-Lonidamine) is an orally active and mitochondria-targeted inhibitor of oxidative phosphorylation (OXPHOS). Mito-LND inhibits mitochondrial bioenergetics, stimulates the formation of reactive oxygen species, and induces autophagic cell death in lung cancer cells. Mito-LND Chemical Structure
  25. GC31682 Mito-TEMPO Mito-Tempo is a mitochondria-targeted antioxidant with effective superoxide scavenging properties Mito-TEMPO Chemical Structure
  26. GC44198 MitoA MitoA is a ratiometric mass spectrometry probe that can be used for assessing changes in H2S within mitochondria in vivo. MitoA Chemical Structure
  27. GC44199 MitoB MitoB is a ratiometric mass spectrometry probe that can be used for assessing changes in H2O2 within mitochondria in vivo. MitoB Chemical Structure
  28. GC61634 Mitochondrial respiration-IN-1 hydrobromide Mitochondrial respiration-IN-1 hydrobromide (compound 49) is a potent mitochondrial inhibitor (IC50=8.8 mg/mL) extracted from patent US20110301180A1, compound 49. Mitochondrial respiration-IN-1 hydrobromide Chemical Structure
  29. GC31123 Mitochonic acid 5 (MA-5) Mitochonic acid 5 (MA-5) binds mitochondria and ameliorates renal tubular and cardiac myocyte damage. Mitochonic acid 5 (MA-5) Chemical Structure
  30. GC44201 MitoP MitoP is a phenol product produced by the reaction of H2O2 with the ratiometric mass spectrometry probe MitoB. MitoP Chemical Structure
  31. GC44202 MitoPBN MitoPBN is a mitochondria-targeted antioxidant. MitoPBN Chemical Structure
  32. GC44203 MitoPerOx MitoPerOx is a ratiometric fluorescent probe that can be used to assess changes in lipid peroxidation within mitochondria. MitoPerOx Chemical Structure
  33. GC44204 MitoPQ

    MitoPQ is comprised of a triphenylphosphonium lipophilic cation conjugated to the redox cycler paraquat.

     MitoPQ Chemical Structure
  34. GC44205 Mitoquinol

    Mitoquinol is a ubiquinone derivative that specifically accumulates in mitochondria due to the covalent attachment of the cation triphenylphosphonium.

     Mitoquinol Chemical Structure
  35. GC49222 Mitoquinol-d15 An internal standard for the quantification of mitoquinol Mitoquinol-d15 Chemical Structure
  36. GC36619 MitoTam bromide, hydrobromide MitoTam bromide, hydrobromide, a Tamoxifen derivative, is an electron transport chain (ETC) inhibitor. MitoTam bromide, hydrobromide reduces mitochondrial membrane potential in senescent cells and affects mitochondrial morphology. MitoTam bromide, hydrobromide is an effective anticancer agent, suppresses respiratory complexes (CI-respiration) and disrupts respiratory supercomplexes (SCs) formation in breast cancer cells. MitoTam bromide, hydrobromide Chemical Structure
  37. GC36620 MitoTam iodide, hydriodide MitoTam iodide, hydriodide is a Tamoxifen derivative, an electron transport chain (ETC) inhibitor, spreduces mitochondrial membrane potential in senescent cells and affects mitochondrial morphology.MitoTam iodide, hydriodide is an effective anticancer agent, suppresses respiratory complexes (CI-respiration) and disrupts respiratory supercomplexes (SCs) formation in breast cancer cells. MitoTam iodide, hydriodide causes apoptosis. MitoTam iodide, hydriodide Chemical Structure
  38. GC44206 MitoTEMPO (hydrate)

    MitoTEMPO is a mitochondria-targeted superoxide dismutase mimetic that possesses superoxide and alkyl radical scavenging properties.

     MitoTEMPO (hydrate) Chemical Structure
  39. GC44207 MitoTEMPOL MitoTEMPOL is a mitochondria-targeting superoxide dismutase mimetic that reduces mitochondrial O2- to H2O2. MitoTEMPOL Chemical Structure
  40. GC18188 MPP+ Iodide

    MPP+ Iodide (1-methyl-4-phenylpyridinium iodide) is a toxic metabolite of the neurotoxin MPTP, and has successfully induced Parkinson-like syndromes in an in vitro model by selectively destroying dopaminergic neurons in substantia nigra.

     MPP+ Iodide Chemical Structure
  41. GC66401 MPP+-d3(iodide) MPP+-d3(iodide) is deuterium labeled MPP+ (iodide). MPP+ iodide, a toxic metabolite of the neurotoxin MPTP, causes symptom of Parkinson's disease in animal models by selectively destroying dopaminergic neurons in substantia nigra. MPP+ iodide is taken up by the dopamine transporter into dopaminergic neurons where it exerts its neurotoxic action on mitochondria by affecting complex I of the respiratory chain. MPP+ iodide is also a high affinity substrate for the serotonin transporter (SERT). MPP+-d3(iodide) Chemical Structure
  42. GC36658 MSDC-0602 MSDC-0602 (MSDC-0602), a PPARγ-sparing thiazolidinedione (TZD), interacts with the mitochondrial pyruvate carrier (MPC) and inhibits its activity and has the potential for type 2 diabetes study with reducing risk of PPARγ-mediated side effects. MSDC-0602 Chemical Structure
  43. GC48399 MTP 131 (acetate) A mitochondria-targeted peptide antioxidant MTP 131 (acetate) Chemical Structure
  44. GC48526 MXS A fluorescent probe for hypochlorous acid MXS Chemical Structure
  45. GC49399 N-Desbutyl Dronedarone (hydrochloride) Debutyldronedarone (SR35021) hydrochloride, the main metabolite of Dronedarone, is a selective thyroid hormone receptor α1 (TRα1) inhibitor. N-Desbutyl Dronedarone (hydrochloride) Chemical Structure
  46. GC44416 N-methyl Mesoporphyrin IX A ferrochelatase inhibitor and turn-on biosensor for DNA N-methyl Mesoporphyrin IX Chemical Structure
  47. GC47789 N-Oleoyl Glutamine An endogenous N-acyl amine N-Oleoyl Glutamine Chemical Structure
  48. GC44441 N-Oleoyl Leucine N-Oleoyl leucine is an N-acyl amide generated by PM20D1 that uncouples mitochondrial respiration independent of uncoupling protein 1 (UCP1) in vitro. N-Oleoyl Leucine Chemical Structure
  49. GC44444 N-Oleoyl Valine N-Oleoyl valine is an endogenous N-acyl amine that acts as an antagonist at the transient receptor potential vanilloid type 3 (TRPV3) receptor, which is involved in thermoregulation. N-Oleoyl Valine Chemical Structure
  50. GC47739 NADH (sodium salt hydrate) A reduced form of NAD+ NADH (sodium salt hydrate) Chemical Structure
  51. GC44308 NADP+ (sodium salt hydrate) NADP+ (sodium salt hydrate), a β-Nicotinamide adenine dinucleotide phosphate sodium salt, is a redox cofactor. NADP+ (sodium salt hydrate) Chemical Structure
  52. GC47747 Napyradiomycin A1 A fungal metabolite with diverse biological activities Napyradiomycin A1 Chemical Structure
  53. GC40669 Nerol Nerol is a monoterpene and isomer of geraniol that has been found in a variety of plants, including Cannabis. Nerol Chemical Structure
  54. GC49511 Nicorandil N-oxide A metabolite of nicorandil Nicorandil N-oxide Chemical Structure
  55. GC52214 Nicotinamide riboside-d4 (triflate)

    An internal standard for the quantification of nicotinamide riboside

     Nicotinamide riboside-d4 (triflate) Chemical Structure
  56. GC31646 NIM811 ((Melle-4)cyclosporin) NIM811 ((Melle-4)cyclosporin) ((Melle-4)cyclosporin; SDZ NIM811 ((Melle-4)cyclosporin)) is an orally bioavailable mitochondrial permeability transition and cyclophilin dual inhibitor, which exhibits potent in vitro activity against hepatitis C virus (HCV) . NIM811 ((Melle-4)cyclosporin) Chemical Structure
  57. GC39411 NL-1 NL-1 is a mitoNEET inhibitor with antileukemic effect. NL-1 inhibits REH and REH/Ara-C cells growth with IC50s of 47.35 ?M and 56.26 ?M, respectively. NL-1-mediated death in leukemic cells requires the activation of the autophagic pathway. NL-1 Chemical Structure
  58. GC12090 Nonactin

    Monovalent cation ionophore that displays selectivity for K+ and NH4+

     Nonactin Chemical Structure
  59. GC52223 Nonadecanoyl-L-carnitine (chloride) A long-chain acylcarnitine Nonadecanoyl-L-carnitine (chloride) Chemical Structure
  60. GC52236 Nonadecanoyl-L-carnitine-d3 (chloride) An internal standard for the quantification of nonadecanoyl-L-carnitine Nonadecanoyl-L-carnitine-d3 (chloride) Chemical Structure
  61. GC44464 NSC 109555 An inhibitor of Chk2 NSC 109555 Chemical Structure
  62. GC44488 Octanoyl Coenzyme A (sodium salt) Octanoyl coenzyme A (octanoyl-CoA) is a medium-chain acyl CoA and a metabolic intermediate in mitochondrial fatty acid β-oxidation. Octanoyl Coenzyme A (sodium salt) Chemical Structure
  63. GC49457 Oleoyl-Coenzyme A (sodium salt)

    An acyl-CoA thioester

     Oleoyl-Coenzyme A (sodium salt) Chemical Structure
  64. GC44506 Oligomycin D

    Oligomycin D is a macrolide antibiotic produced by several species of Streptomyces that inhibits the mitochondrial F1FO-ATPase and is used to uncouple oxidative phosphorylation from electron transport.

     Oligomycin D Chemical Structure
  65. GC47868 Palmitoyl-L-carnitine-d3 (chloride) An internal standard for the quantification of palmitoyl-L-carnitine Palmitoyl-L-carnitine-d3 (chloride) Chemical Structure
  66. GC52306 Pentadecanoyl-L-carnitine (chloride) A CB receptor agonist Pentadecanoyl-L-carnitine (chloride) Chemical Structure
  67. GC40252 Phosphatidic Acids (egg) (ammonium salt) Phosphatidic acid is a phospholipid and an intermediate in glycerolipid biosynthesis. Phosphatidic Acids (egg) (ammonium salt) Chemical Structure
  68. GC18522 Phosphatidylserines (sodium salt) Phosphatidylserine is a naturally occurring phospholipid that comprises 2-10% of total phospholipids in mammals and is enriched in the central nervous system, particularly the retina. Phosphatidylserines (sodium salt) Chemical Structure
  69. GC48528 Piericidin B A bacterial metabolite with insecticidal and antimicrobial activities Piericidin B Chemical Structure
  70. GC49192 Piracetam-d6 An internal standard for the quantification of piracetam Piracetam-d6 Chemical Structure
  71. GC47980 Propionyl-L-carnitine-d3 (chloride)

    An internal standard for the quantification of propionyl-L-carnitine

     Propionyl-L-carnitine-d3 (chloride) Chemical Structure
  72. GC48030 RC574 An inhibitor of ferroptosis RC574 Chemical Structure
  73. GC16775 Rotenone

    inhibitor of the mitochondrial complex I electron transport chain

     Rotenone Chemical Structure
  74. GC60330 Ru360 Ru360 Chemical Structure
  75. GC64669 RYL-552 RYL-552, a mitochondrial electron transport chain (ETC) inhibitor, is a P. RYL-552 Chemical Structure
  76. GC44860 S1QEL1.1 S1QEL1.1 is a small molecule that suppresses superoxide production during reverse electron transport through the IQ site of the mitochondrial respiratory complex I (IC50 = 70 nM) without affecting oxidative phosphorylation. S1QEL1.1 Chemical Structure
  77. GC44862 S3QEL-2

    S3QEL-2 is a cell-permeable, selective suppressor of electron leak from mitochondrial respiratory complex III, inhibiting site IIIQo superoxide production (IC50 = 1.7 μM) without altering normal bioenergetics function.

     S3QEL-2 Chemical Structure
  78. GC48547 Sartorypyrone D A fungal metabolite Sartorypyrone D Chemical Structure
  79. GN10091 Schaftoside Schaftoside Chemical Structure
  80. GC30459 Senecionine (Senecionan-11,16-dione, 12-hydroxy-) Senecionine (Senecionan-11,16-dione, 12-hydroxy-) (Senecionan-11,16-dione, 12-hydroxy-) is a pyrrolizidine alkaloid isolated from Senecio vulgaris. Senecionine (Senecionan-11,16-dione, 12-hydroxy-) Chemical Structure
  81. GC61274 Senecionine acetate Senecionine acetate (O-Acetylsenecionine) is a pyrrolizidine alkaloid. Senecionine acetate Chemical Structure
  82. GC48077 SHS4121705 A mitochondrial uncoupler SHS4121705 Chemical Structure
  83. GC48894 SIH SIH is an antituberculous compound. SIH Chemical Structure
  84. GC31491 Speract

    Speract, a sea urchin egg peptide that regulates sperm motility, also stimulates sperm mitochondrial metabolism.

     Speract Chemical Structure
  85. GC63669 T0467 T0467 activates parkin mitochondrial translocation in a PINK1-dependent manner in vitro. T0467 Chemical Structure
  86. GC39512 Tempo Tempo is a classic nitroxide radical and is a selective scavenger of ROS that dismutases superoxide in the catalytic cycle. Tempo induces DNA-strand breakage. Tempo can be used as an organocatalyst for the oxidation of primary alcohols to aldehydes. Tempo has mutagenic and antioxidant effects. Tempo Chemical Structure
  87. GC45020 Tetrachlorohydroquinone Tetrachlorohydroquinone (TCHQ) is a metabolite of the organochlorine biocide pentachlorophenol. Tetrachlorohydroquinone Chemical Structure
  88. GC48924 TFM A piscicide TFM Chemical Structure
  89. GC17228 Thiabendazole Fungicide Thiabendazole Chemical Structure
  90. GC64688 THP104c THP104c is a mitochondrial fission inhibitor. THP104c Chemical Structure
  91. GC49529 Thymidine-d4 An internal standard for the quantification of thymidine Thymidine-d4 Chemical Structure
  92. GC48192 trans-2-Octenoyl-L-carnitine A synthetic carnitine trans-2-Octenoyl-L-carnitine Chemical Structure
  93. GC63238 Tricarballylic acid Tricarballylic acid, a conjugate acid of a?tricarballylate, is a competitive inhibitor of the enzyme aconitate hydratase (aconitase; EC 4.2.1.3) with a Ki value of 0.52 mM. Tricarballylic acid Chemical Structure
  94. GC13726 TRO 19622 TRO 19622 (TRO 19622) is a mitochondrial-targeted neuroprotective compound with mean EC50 value for increasing cell survival is 3.2±0.2 μM. TRO 19622 Chemical Structure
  95. GC11865 UK-5099 An inhibitor of the mitochondrial pyruvate carrier UK-5099 Chemical Structure
  96. GC30512 Vatiquinone (EPI-743) Vatiquinone (EPI-743) (Vatiquinone; α-Tocotrienol quinone; PTC-743; NCT04378075) is a potent cellular oxidative stress protectant, inhibits ferroptosis in cells, which could be used for the study for mitochondrial diseases. Vatiquinone (EPI-743) Chemical Structure
  97. GC48398 WST-1 A water-soluble and cell-permeable tetrazolium dye WST-1 Chemical Structure

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