8 Results for "

propionic acidemia

" in MedChemExpress (MCE) Product Catalog:
Products (8)

8 Results for "propionic acidemia" in MCE Product Catalog:

Cat. No.: HY-113371
CAS No.: 6061-96-7
Purity:  99.70%
Synonyms: Methylcitric acid
2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate .
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Cat. No.: HY-159509
CAS No.: 2361124-03-8
Synonyms: Claziprotamide; BBP-671
Target:  

PANK

Claziprotamidum (Claziprotamide; BBP-671) is a panthothenate kinase (PANK) activator with oral activity and blood-brain barrier permeability. Claziprotamidum binds to and activates all PANK isoforms, with the highest affinity for PANK3 (KD = 97 pM; IC50 = 1.39 nM). Claziprotamidum increases the intracellular biosynthesis of Coenzyme A (HY-128851) and restores mitochondrial function. Claziprotamidum can be used in the research of pantothenate kinase-associated neurodegeneration and propionic acidemia .
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Cat. No.: HY-113371A
CAS No.: 117041-96-0
Purity:  ≥96.0%
Synonyms: Methylcitric acid trisodium
2-Methylcitric acid trisodium (Methylcitric acid trisodium) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid trisodium accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid trisodium markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate .
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Cat. No.: HY-164909
CAS No.: 2170608-85-0
Target:  

PANK

Research Areas:  

Metabolic Disease

PZ-3022 is an orally active allosteric agonist of pantothenate kinase (PanK) with an EC50 of 5.3 nM against PanK3. PZ-3022 antagonizes the inhibitory effect of C3-CoA. PZ-3022 increases CoA levels in cells and the liver, upregulates CoASH and C2-CoA, downregulates C3-CoA, and restores impaired TCA cycle and mitochondrial function. PZ-3022 can be used for the research of propionic acidemia and metabolic CoA deficiency .
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Cat. No.: HY-113406
CAS No.: 6418-78-6
Synonyms: DL-Hexanoylcarnitine
(±)-Hexanoylcarnitine exists in human urine and plasma. (±)-Hexanoylcarnitine can be used as a plasma detection indicator in patients with methylmalonic aciduria, propionic acidemia, and medium-chain acyl-CoA dehydrogenase deficiency .
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Cat. No.: HY-113371S
CAS No.: 146764-58-1
Purity:  98.90%
Synonyms: Methylcitric acid-d3
2-Methylcitric acid-d3 is the deuterium labeled 2-Methylcitric acid. 2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate .
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Cat. No.: HY-P1162
CAS No.: 73168-24-8
Synonyms: SKF 100273
Target:  

Vasopressin Receptor

Research Areas:  

Metabolic Disease

(d(CH2)51,Tyr(Me)2,Arg8)-Vasopressin (SKF 100273) is a vasopressin V1 receptor selective antagonist .
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Cat. No.: HY-P2595
CAS No.: 111372-60-2
Target:  

Endogenous Metabolite

Research Areas:  

Cardiovascular Disease

SKF 103784 is an vasopressin antagonist with activity against vasopressin. SKF 103784 inhibits the physiological response caused by antidiuretic and is therefore used to study biological processes related to water and salt balance. SKF 103784 can also be used to explore pathological mechanisms related to cardiovascular diseases and endocrine dysfunction .
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