Mitochondrial Metabolism

MitoSOX Red is a novel fluorescent probe that is specific and cell membrane-permeable and targets mitochondria in living cells.MitoSOX Red is oxidized by superoxide upon entry into the mitochondria and is not oxidized by other ROS or RNS-generating systems.MitoSOX Red then binds to nucleic acids in the mitochondrion and produces a strong red fluorescence.MitoSOX Red can be used as a fluorescent indicator for the specific detection of superoxide.MitoSOX Red can be used as a fluorescent indicator for the specific detection of superoxide. MitoSOX Red can be used as a fluorescent indicator to specifically detect superoxide. Superoxide dismutase (SOD) prevents the oxidation of MitoSOX Red.

Imeglimin hydrochloride (EMD 387008 hydrochloride） is an oral hypoglycemic agent. Imeglimin improves insulin sensitivity, inhibits the production of reactive oxygen species, increases mitochondrial DNA, and improves mitochondrial function.

Mito-TEMPO, a mitochondria-targeted superoxide dismutase mimetic, scavenges superoxide and alkyl radicals, preventing mitochondrial oxidation, necrosis, and apoptosis.

IMT1 (Propanamide, N,N-dimethyl-2-[[4-(2-methylphenyl)-2-oxo-2H-1-benzopyran-7-yl]oxy]-) has anticancer activity.

Schaftoside (APIGENIN-6-GLUCOSIDE-8-ARABINOSIDE) has antioxidant and anticancer activity.

CCCP (Carbonyl Cyanide m-Chlorophenylhydrazone) is an oxidative phosphorylation (OXPHOS) inhibitor and mitochondrial proton carrier uncoupler. CCCP inhibits the activation of STING and its downstream signaling molecules TBK1 and IRF3.

MPP+ iodide, the metabolite of a neurotoxin MPTP, causes symptom of Parkinson's disease (PD) in animal models by selectively destroying dopaminergic neurons in substantia nigra. MPP+ induces dopamine transporter (DAT) externalization in dopaminergic (DA) neurons, but internalization of serotonin transporter (SERT) in serotonergic (5-HT) neurons. MPP+ induces autophagic cell death in SH-SY5Y cells.

FCCP (Trifluoromethoxy carbonylcyanide phenylhydrazone) is an oxidative phosphorylation (OXPHOS) inhibitor and mitochondrial proton carrier uncoupler. FCCP is often used as an apoptosis inducer.

McN3716 is a carnitine palmitoyltransferase I (CPT-1) inhibitor used for researching metabolic diseases.

UK-5099 (PF-1005023) is a specific and potent inhibitor of MPC carrier activity, effectively inhibiting pyruvate-dependent oxygen consumption in rat heart mitochondria with an IC50 of 50 nM [1].

KL1333 (NQO1 activator 1) enhances energy metabolism and mitochondrial dysfunction in MELAS fibroblasts. Elevated NAD+ levels trigger the activation of SIRT1 and AMPK, and subsequently activates PGC-1α. NQO1 activator 1 is an orally available NAD+ modulator. It reacts with NAD(P)H: NQO1 as a substrate, resulting in increases in intracellular NAD+ levels via NADH oxidation.

Perhexiline maleate is an orally active inhibitor of CPT1 and CPT2, reducing fatty acid metabolism, with IC50 values of 77 μM and 148 μM against rat heart and liver CPT1, respectively.

MFI8 is a compound that regulates mitochondrial fission and can be used to study aging.

IACS-010759 is an orally bioavailable inhibitor of complex I of oxidative phosphorylation of the mitochondrial electron transport chain.

Gboxin is an inhibitor of oxidative phosphorylation that targets glioblastoma,inhibits the activity of F0F1 ATP synthase, with antitumour activity.

Devimistat (6,8-Bis(benzylthio)octanoic acid) , a lipoate analog, inhibits mitochondrial enzymes pyruvate dehydrogenase (PDH) and α-ketoglutarate dehydrogenase, disrupts tumor cell mitochondrial metabolism. It has potential chemopreventive and antineoplastic activities, and has been used in trials studying the treatment of Cancer, Lymphoma, Solid Tumors, Advanced Cancer, and Pancreatic Cancer, among others.

Daidzin (Daidzoside) is an isoflavone isolated from soya bean with anti-oxidant, anti-carcinogenic, and anti-atherosclerotic activities.

HQNO is a natural product isolated from P. aeruginosa,and is a potent inhibitor of electron transport chain(Kd of 64 nM for complex III). It also is a potent mitochondrial NDH-2 inhibitorin many species.

BRD6897 is a mitochondrial content inducer that increases the cellular content of mitochondria and induces a significant increase in the electron density of existing mitochondria.

7ACC2 is a new potent MCT inhibitor with IC50 of 11 nM for [14C]-lactate influx; new antitumor treatment targeting lactate transport in Y cells.

Dihydrorotenone is a potent mitochondrial inhibitor and probably induces Parkinsonian syndrome.

GW604714X (2,4-Thiazolidinedione, 5-[[5-[6-(4-acetyl-1-piperazinyl)-3-nitro-2-pyridinyl]-2-fluorophenyl]methylene]-) were found to be potent inhibitors of mitochondrial respiration supported by pyruvate

Mitochondrial fusion promoter M1, a modulator of mitochondrial dynamics, enhances mitochondrial function and cellular respiration, and has shown efficacy in reducing brain and cardiac damage in rats suffering from cardiac ischemia/reperfusion injury.

Agaricic acid is an adenine nucleotide translocase antagonist.It is obtained from various plants of the fungous tribe, i.e. Polyporus officinalis and Polyporus igniarius. Agaric acid (Agaricinic Acid) induces mitochondrial permeability transition through its interaction with the adenine nucleotide translocase. Agaric acid promotes efflux of accumulated Ca 2+, collapse of transmembrane potential, and mitochondrial swelling. Agaric acid is used to regulate lipid metabolism.

Adjudin (AF-2364) is a small molecule compound known to possess antispermatogenic function, attenuates microglia activation by suppression of the NF-κB pathway.

Glibenclamide (Glyburide) is an antidiabetic sulfonylurea derivative with actions similar to those of chlorpropamide.

BAM 15 is an uncoupler of mitochondrial protonophore.

Idebenone (CV-2619) is a synthetic analogue of ubiquinone (Coenzyme Q10), a vital cell antioxidant and essential component of the Electron Transport Chain (ETC).

MCU-i4 is a novel negative modulator of the MCU, binding MICU1 and impairing muscle cell growth.

Mito-LND (Mito-Loidamine) is an orally active and mitochondria-targeted inhibitor of oxidative phosphorylation.

Mitochonic acid 5 (MA-5) is a derivative of the plant hormone indole-3-acetic acid. Mitochonic acid 5 binds mitochondria and ameliorates renal tubular and cardiac myocyte damage.It has been shown to improve survival of fibroblasts from patients with mitochondrial diseases.

Fumarate hydratase-IN-1 is a cell-permeable fumarate hydratase inhibitor with antiproliferative activity and can be used to study cellular activity.

ER-000444793 is a potent mitochondrial permeability transition pore (mPTP) opening inhibitor and it also inhibits mPTP (IC50: 2.8 μM).

Lonidamine (Diclondazolic Acid) is an indazole carboxylic acid derivative, principally inhibiting aerobic glycolytic activity, by its effect on mitochondrially-bound hexokinase (HK).

NL-1, a mitoNEET inhibitor, exhibits antileukemic effects.

Afzelin (Kaempferol-3-O-rhamnoside) exhibits several cellular activities such as DNA-protective, antibacterial, antioxidant, anti-inflammatory, and UV-absorbing properties, potentially protecting human skin from UVB-induced damage through a combination of UV-absorbing and cellular actions. Afzelin mitigates mitochondrial damage, promotes mitochondrial biogenesis, and reduces levels of mitophagy-related proteins, parkin and PTEN-induced putative kinase 1.

CTPI-2 is an inhibitor of mitochondrial citrate carrier SLC25A1 with(KD : 3.5 μM). CTPI-2 inhibits glycolysis, PPARγ, and its downstream target the glucose transporter GLUT4. CTPI-2 exhibits anti-tumor activity.CTPI-2 halts salient alterations of NASH reverting steatosis, preventing the evolution to steatohepatitis, reducing inflammatory macrophage infiltration in the liver and adipose tissue, and starkly mitigating obesity induced by a high-fat diet.

Thiabendazole (2-(4-Thiazolyl)benzimidazole) is a benzimidazole derivative with anthelminthic property.

THP104c is an inhibitor of mitochondrial fission.

VLX600 is an iron-chelating oxidative phosphorylation (OXPHOS) inhibitor, is a cell-permeable anticancer agent. It acts by reducing mitochondrial oxidative phosphorylation in tumor cells.

Kresoxim-methyl is a strobilurin fungicide,inhibits conidial germination of V. inaequalis isolates from apple orchards .

Tebufenpyrad (Comanche) (mitochondrial complex-1 inhibitors) is an agro-chemically important acaricide that functions like the known mitochondrial toxicant rotenone.

Glutamate dehydrogenase NAD(P) (GLDH), a mitochondrial enzyme found in hepatocytes, renal tissue, brain, muscle, and intestinal cells, catalyzes the reversible oxidative deamination of glutamate to α-ketoglutarate (α-KG), playing a critical role in the urea cycle [1], and is frequently utilized in biochemical research.

Tempo (2,2,6,6-Tetramethylpiperidinooxy) has a wide range of applications including use as a free radical scavenger, a reagent in organic synthesis and as a structural probe in electron spin resonance spectroscopy.

Genistein 8-c-glucoside (G8CG), a natural glucoside isolated from the flowers of Lupinus luteus L., induces mitochondrial membrane depolarization and apoptosis.

Imeglimin (EMD 387008) is an orally available antidiabetic compound that enhances mitochondrial function, enhances insulin secretion, promotes β-cell proliferation and improves pancreatic β-cell survival in mice.

S3QEL-2 is a selective complex III superoxide-producing site inhibitor with neuroprotective effects that inhibits PC-3 cell proliferation in pyruvate-starved medium in a dose-dependent manner.

L-2-Hydroxyglutaric acid disodium ((S)-2-Hydroxyglutaric acid disodium) is an epigenetic modifier and putative oncometabolite in renal cancer. L-2-Hydroxyglutaric acid inhibits mitochondrial creatine kinase (Mi-CK) activity(Km and Ki of 2.52 mM and 11.13 mM, respectively).