75 Results for "

mitochondrial disorders

" in MedChemExpress (MCE) Product Catalog:
Products (75)

75 Results for "mitochondrial disorders" in MCE Product Catalog:

12
12 Publications Verification
Cat. No.: HY-134539
CAS No.: 2304621-31-4
Purity:  98.13%
IMT1 is a first-in-class specific and noncompetitive human mitochondrial RNA polymerase (POLRMT) inhibitor. IMT1 causes a conformational change of POLRMT, which blocks substrate binding and transcription in a dose-dependent way in vitro. IMT1 reduces deoxynucleoside triphosphate levels and citric acid cycle intermediates, resulting in a marked depletion of cellular amino acid levels. IMT1 has the potential for mitochondrial transcription disorders related diseases .
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11
11 Cited Publications
Cat. No.: HY-153169
CAS No.: 2754428-18-5
Purity:  99.30%
Synonyms: 6PPD-Quinone
6PPD-Q (6PPD-Quinone) is an environmental pollutant that can be detected in human urine and is widely present in the environment. 6PPD-Q targets and binds to CNR2, CNR1, AA2AR, LCAT, and TRPA1, with CNR2 exhibiting the highest binding affinity, potentially acting as a CNR2 receptor agonist to activate cannabinoid receptors. 6PPD-Q induces intestinal inflammation and barrier damage by disrupting mitochondrial function, reducing neuronal glycolysis metabolites and TCA cycle intermediates, and exacerbating α-synuclein (α-syn) aggregation. 6PPD-Q is applicable in research on environmental toxicology, neurodegenerative diseases, and inflammation-related disorders .
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6
6 Cited Publications
Cat. No.: HY-N9933
CAS No.: 25696-60-0
Synonyms: TβMCA
Tauro-β-muricholic acid (TβMCA) is an orally active trihydroxylated bile acid and a competitive, reversible FXR antagonist (IC50=40 μM). Tauro-β-muricholic acid inhibits bile acid-induced hepatocyte apoptosis by maintaining mitochondrial membrane potential, while simultaneously inhibiting intestinal FXR signaling, affecting bile acid synthesis, hepatic lipid metabolism, and insulin sensitivity. Accumulation of tauro-β-muricholic acid disrupts metabolic homeostasis, promoting cancer stem cell proliferation and tumor progression. The mechanisms of tauro-β-muricholic acid involve two aspects: first, inhibiting the translocation of the pro-apoptotic protein Bax to mitochondria and maintaining mitochondrial membrane potential (MMP); and second, blocking the FXR signaling pathway to regulate bile acid metabolism, reduce serum ceramide production, and downregulate the hepatic SREBP1C/CIDEA pathway. Tauro-β-muricholic acid possesses anti-hepatocyte apoptosis, bile acid homeostasis regulation, and liver fat accumulation reduction properties, and also functions as a biomarker, making it useful in the study of diseases such as bile acid metabolism disorders, non-alcoholic fatty liver disease, colorectal cancer, and liver fibrosis .
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6
6 Cited Publications
Cat. No.: HY-N0430A
CAS No.: 1198398-71-8
Coptisine Sulfate is an orally active and brain-penetrant alkaloid found in Coptis chinensis. Coptisine Sulfate is a reversible, uncompetitive IDO inhibitor with a Ki of 5.8 μM and an IC50 of 6.3 μM. Coptisine Sulfate suppresses neuroinflammation, reduces Aβ plaque burden and shows neuroprotective activity. Coptisine Sulfate shows anti-inflammation activity by blocking NF-κB, MAPK, and PI3K/Akt activation. Coptisine Sulfate inhibits cancer cells proliferation, induces DNA damage, G2/M phase cell cycle arrest, apoptosis, ROS production and mitochondrial dysfunction. Coptisine Sulfate inhibits Rho/ROCK pathway activation, reduces arrhythmia, limits cardiac injury marker release, reduces infarct size, and preserves cardiac function in rat myocardial ischemia/reperfusion models. Coptisine Sulfate downregulates HMGCR and upregulates LDLR and CYP7A1 to modulate cholesterol metabolism, reduces abnormal serum lipid levels, and promotes fecal bile acid excretion. Coptisine Sulfate be used for the research of cancer, hypercholesterolemia, Alzheimer’s disease, inflammatory disorders and cardiovascular disease .
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5
5 Cited Publications
Cat. No.: HY-N5034
CAS No.: 1071-23-4
Synonyms: Monoaminoethyl phosphate; NSC 254167; O-Phosphoethanolamine
Phosphorylethanolamine (Monoaminoethyl phosphate) is a membrane phospholipid and an important precursor of Phosphatidylcholine (HY-N18891). It is found in most animal tissues and various human extracranial tumors, playing a critical role in membrane integrity, cell division, mitochondrial respiratory function, and more. Studies have shown that changes in the abundance of Phosphorylethanolamine are associated with Alzheimer's disease and Parkinson's disease. Lowering the ratio of Phosphorylethanolamine to Phosphatidylcholine in the liver can improve insulin signaling. Phosphorylethanolamine holds promise for research in the fields of cancer, neurodegenerative disorders, and metabolic diseases .
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4
4 Cited Publications
Cat. No.: HY-N0157
CAS No.: 65-86-1
Synonyms: 6-Carboxyuracil; Vitamin B13
Orotic acid (6-Carboxyuracil), a precursor in biosynthesis of pyrimidine nucleotides and RNA, is released from the mitochondrial dihydroorotate dehydrogenase (DHODH) for conversion to UMP by the cytoplasmic UMP synthase enzyme. Orotic acid is a marker for measurement in routine newborn screening for urea cycle disorders. Orotic acid can induce hepatic steatosis and hepatomegaly in rats .
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4
4 Cited Publications
Cat. No.: HY-N0157A
CAS No.: 68399-76-8
Synonyms: 6-Carboxyuracil zinc; Vitamin B13 zinc
Orotic acid (zinc), a precursor in biosynthesis of pyrimidine nucleotides and RNA, is released from the mitochondrial dihydroorotate dehydrogenase (DHODH) for conversion to UMP by the cytoplasmic UMP synthase enzyme. Orotic acid (zinc) is a marker for measurement in routine newborn screening for urea cycle disorders. Orotic acid (zinc) can induce hepatic steatosis and hepatomegaly in rats .
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3
3 Cited Publications
Cat. No.: HY-124410
CAS No.: 845959-55-9
Purity:  98.00%
Mitoquinol is an orally active mitochondria-targeted antioxidant. Mitoquinol can regulate mitochondrial respiration and oxidation. Mitoquinol inhibits ROS production, and improves phagocytosis and glycolysis in ethanol-exposed macrophages via the HIF-1α-PFKP axis. Additionally, Mitoquinol can partially alleviate heat stress-induced decreases in growth performance, inflammatory responses, and metabolic disorders in pigs .
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3
3 Cited Publications
Cat. No.: HY-139308
CAS No.: 859518-94-8
Purity:  99.30%
T0467 activates parkin mitochondrial translocation in a PINK1-dependent manner in vitro. T0467 do not induce mitochondrial accumulation of PINK1in dopaminergic neurons. T0467 is a potential compound for PINK1-Parkin signaling activation, and can be used for parkinson's disease and related disorders research .
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2
2 Cited Publications
Cat. No.: HY-B0762
CAS No.: 5080-50-2
Synonyms: O-Acetyl-L-carnitine hydrochloride; ALCAR hydrochloride
Target:  

Caspase Apoptosis

Acetyl-L-carnitine (O-Acetyl-L-carnitine; ALCAR) hydrochloride is an orally active mitochondrial energy metabolism regulator and neuroprotectant that can penetrate the blood-brain barrier. Acetyl-L-carnitine hydrochloride selectively enters cells and the brain through the organic cation transporter OCTN2. Acetyl-L-carnitine hydrochloride can participate in fatty acid β-oxidation, promote acetylcholine synthesis, regulate mitochondrial function and inhibit oxidative stress as an acetyl donor. Acetyl-L-carnitine hydrochloride exerts its activity by enhancing energy metabolism, protecting neurons and improving synaptic plasticity. Acetyl-L-carnitine hydrochloride is mainly used in the study of neurodegenerative diseases and metabolic disorder-related diseases such as neonatal hypoxic-ischemic brain damage, Alzheimer's disease, and depression .
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2
2 Cited Publications
Cat. No.: HY-113218
CAS No.: 3040-38-8
Purity:  ≥98.0%
Synonyms: O-Acetyl-L-carnitine; ALCAR
Target:  

Caspase Apoptosis

Acetyl-L-carnitine (O-Acetyl-L-carnitine; ALCAR) is an orally active mitochondrial energy metabolism regulator and neuroprotectant that can penetrate the blood-brain barrier. Acetyl-L-carnitine selectively enters cells and the brain through the organic cation transporter OCTN2. Acetyl-L-carnitine can participate in fatty acid β-oxidation, promote acetylcholine synthesis, regulate mitochondrial function and inhibit oxidative stress as an acetyl donor. Acetyl-L-carnitine exerts its activity by enhancing energy metabolism, protecting neurons and improving synaptic plasticity. Acetyl-L-carnitine is mainly used in the study of neurodegenerative diseases and metabolic disorder-related diseases such as neonatal hypoxic-ischemic brain damage, Alzheimer's disease, and depression .
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2
2 Cited Publications
Cat. No.: HY-N0735
CAS No.: 104112-82-5
Phellodendrine chloride is an orally active plant alkaloid. Phellodendrine chloride inhibits the proliferation of KRAS-mutated pancreatic cancer cells by suppressing macropinocytosis and glutamine metabolism, inducing ROS accumulation and mitochondrial apoptosis. Phellodendrine chloride promotes autophagy by activating the AMPK/mTOR pathway, alleviating intestinal damage in ulcerative colitis. Phellodendrine chloride can alleviate gouty arthritis by inhibiting the IL-6/STAT3 signaling pathway. Phellodendrine chloride suppresses allergic reactions by altering the conformation of MRGPRB3/MRGPRX2 protein, thereby inhibiting the activation of PKC and subsequent downstream MAPK and NF-κB signaling. Phellodendrine chloride inhibits the AKT/NF-κB pathway and down-regulates the expression of COX-2, thereby protecting zebrafish embryos from oxidative stress. Phellodendrine chloride has an anti-major depressive disorder (MDD) effect by down-regulating CHRM1, HTR1A, and the PI3K/Akt signaling pathway .
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2
2 Cited Publications
Cat. No.: HY-P1723
CAS No.: 1370290-58-6
Synonyms: Neuropeptide Q
Spexin (Neuropeptide Q) is a selective agonist of galanin receptors GAL2 and GAL3, and is a conserved peptide that functions as a neurotransmitter/neuromodulator and endocrine factor. Spexin can function through both central and peripheral actions. Spexin upregulates Beclin 1 to inhibit ferroptosis induced by excessive autophagy, reduces the uptake of long-chain fatty acids by adipocytes, and regulates energy metabolism by increasing lipid oxidation (e.g., reducing the respiratory exchange ratio in rodents). Spexin improves cardiac function in the Doxorubicin hydrochloride (HY-15142)-induced cardiotoxicity model, protects mitochondrial membrane potential, and reduces iron accumulation and lipid peroxidation. Spexin can be used to study obesity and its related metabolic disorders, cardiovascular diseases (e.g., cardioprotection), and side effects of tumor chemotherapy .
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2
2 Cited Publications
Cat. No.: HY-128578
CAS No.: 906669-07-6
Purity:  99.86%
KPLH1130 is a pyruvate dehydrogenase kinase (PDK) inhibitor. KPLH1130 potently inhibits M1 macrophage polarization by reducing the expression of pro-inflammatory cytokines, decreasing the levels of M1 phenotype markers (HIF-1α, iNOS) and nitric oxide (NO) production. KPLH1130 prevents the reduction of mitochondrial oxygen consumption rate (OCR) induced by inflammatory stimuli (LPS ((HY-D1056) + IFN-γ) in various macrophage types. KPLH1130 improves glucose tolerance in HFD-fed mice. KPLH1130 can be used for the study of obesity-associated metabolic disorders and other inflammatory conditions .
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1
1 Cited Publications
Cat. No.: HY-119976
CAS No.: 188425-85-6
Purity:  99.29%
Boscalid is a succinate dehydrogenase (SDHI) inhibitor with antifungal activity. Boscalid binds to the ubiquinone-binding site of fungal mitochondrial complex II, blocks ATP production and aerobic respiration, exhibits good control efficacy against a variety of plant fungal diseases including gray mold, sclerotinia rot and powdery mildew, and is widely used for disease control in agriculture. Boscalid induces apoptosis, altered lipid metabolism, mitochondrial dysfunction, respiratory impairment, oxidative stress, ROS accumulation and neurodevelopmental disorders in zebrafish. Boscalid reduces foraging ability, shortens median death time and causes chronic toxicity in exposed honeybees. Boscalid also possesses genotoxicity, cytotoxicity, elevated mitochondrial superoxide levels and early-stage apoptosis .
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1
1 Cited Publications
Cat. No.: HY-P2048
CAS No.: 1627580-64-6
MOTS-c (human) is a mitochondrial-derived peptide that modulates the AMPK/PGC-1α pathway to enhance insulin sensitivity. MOTS-c (human) inhibits the folate cycle and de novo purine synthesis, increases AICAR levels to activate AMPK, and then regulates the Nrf2/Keap1 antioxidant pathway and inhibits the NF-κB inflammatory pathway, while promoting mitochondrial biogenesis and energy metabolism. MOTS-c (human) has the effects of improving glucose and lipid metabolism, anti-oxidative stress, anti-inflammatory and neuroprotection, and can be used in the study of type 2 diabetes, traumatic brain injury, inflammatory diseases and aging-related metabolic disorders .
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1
1 Cited Publications
Cat. No.: HY-B0863
CAS No.: 1071-83-6
Glyphosate, a non-selective systemic biocide with broad-spectrum activity, is an herbicidal derivative of the amino acid glycine. Glyphosate inhibits the enzymatic activity of the 5-endopyruvylshikimate 3-phosphate synthase (EPSPS) in the shikimic acid pathway, preventing the synthesis of the aromatic amino acids tyrosine, phenylalanine, and tryptophan. Glyphosate induces oxidative stress, neuroinflammation, and mitochondrial dysfunction, processes that lead to neuronal death by autophagia, necrosis, or apoptosis, as well as the appearance of behavioral and motor disorders .
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1
1 Cited Publications
Cat. No.: HY-113166
CAS No.: 25518-54-1
Purity:  99.75%
Synonyms: (-)-Lauroylcarnitine
Dodecanoylcarnitine ((-)-Lauroylcarnitine) is a medium long-chain acylcarnitine, an intermediate product in key energy metabolic pathways of fatty acid β-oxidation and amino acids catabolism. The abnormal decrease in the Dodecanoylcarnitine levels indicats that mitochondrial fuel metabolism, including fatty acid oxidation is significantly disturbed. Changes in plasma concentrations of Dodecanoylcarnitine are not only associated with type II diabetes, but also with pre-diabetes status. Dodecanoylcarnitine is present in fatty acid oxidation disorders such as long-chain acyl CoA dehydrogenase deficiency, carnitine palmitoyltransferase I/II deficiency, and is also associated with celiac disease. Dodecanoylcarnitine deomonstrates high sensitivities and specificities in predicting asthma. Combined model of Decanoylcarnitine (HY-113069), Dodecanoylcarnitine, PC (16:0/0:0), and Asp Arg Pro can be used as a potential biomarker for the diagnosis of Yin-deficiency-heat syndrome .
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1
1 Cited Publications
Cat. No.: HY-W250122
CAS No.: 32221-81-1
Synonyms: Monosodium glutamate
Glutamic acid sodium salt (Monosodium glutamate) is an orally active food flavor enhancer. Glutamic acid sodium salt causes ROS generation, mitochondrial dysfunction, and Apoptosis. Glutamic acid sodium salt upregulates CHOP, Grp78, and Bcl-2. Glutamic acid sodium salt impairs cognition, induces depressive-like behavior, induces hyperalgesia, and induces obesity and insulin resistance. Glutamic acid sodium salt can be used to study neurotoxicity (e.g., brain damage, cognitive impairment), metabolic disorders (e.g., obesity, insulin resistance), hepatotoxicity, and renal toxicity, as well as pain-related disorders .
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1
1 Cited Publications
Cat. No.: HY-142035
CAS No.: 58160-95-5
Purity:  99.70%
N-Propargylglycine is a brain-penetrant and orally active PRODH inhibitor. N-Propargylglycine covalently modifies enzyme-bound FAD and active site lysine, causing enzyme structural distortion, protein decay, and irreversible inhibition of proline and 4-hydroxyproline catabolism. N-Propargylglycine induces UPRmt, upregulates mitochondrial chaperones and YME1L1, enhances mitochondrial proteostasis, blocks astrocytic L-proline consumption, and abolishes L-proline’s ATP-maintaining and viability-protective effects. N-Propargylglycine stimulates neural processes, increases brain proline, hydroxyproline, and sarcosine levels, partially normalizes Huntington’s disease whole brain transcriptomes. N-Propargylglycine reduces hyperoxaluria, prevents calcium oxalate stone formation, reduces kidney tubular damage, and restores weight and survival in Grhpr knockout mice. N-Propargylglycine can be used for the research of breast cancer, neurodegenerative disorders, Huntington’s disease, and primary hyperoxaluria type 2 .
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