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methylmalonic acidemia

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Amino acid Transporter (1) CD38 (1) Endogenous Metabolite (3) Ferroportin (1)
By Research Areas:	Cancer (1) Inflammation/Immunology (1) Metabolic Disease (4)

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Isotope-Labeled Compounds

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-P10272

Rusfertide PTG-300	Ferroportin	Others
Rusfertide is a peptide mimetic of natural hepcidin, which targets and degrades ferroportin, reduces serum iron and transferrin-saturation, and thus regulates the production of red blood cells. Rusfertide ameliorates the polycythemia vera, β-thalassemia and hereditary hemochromatosis .

HY-113371

2-Methylcitric acid Methylcitric acid	Endogenous Metabolite	Metabolic Disease
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 .

HY-113371A

2-Methylcitric acid trisodium Methylcitric acid trisodium	Endogenous Metabolite	Metabolic Disease
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 .

HY-113406

(±)-Hexanoylcarnitine DL-Hexanoylcarnitine	Others	Inflammation/Immunology
(±)-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 .

HY-113371S

2-Methylcitric acid-d3 Methylcitric acid-d₃	Endogenous Metabolite	Metabolic Disease
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 .

HY-158161

SLC6A19-IN-1	Amino acid Transporter	Metabolic Disease
SLC6A19-IN-1 is a potent and highly selective inhibitor and transport corrector of human SLC6A19 (IC₅₀=47 nM). Furthermore, at a concentration of 35 μM, SLC6A19-IN-1 exhibits no activity against SLC1A5, SLC7A5, or SLC6A8. SLC6A19-IN-1 is applicable to research on phenylketonuria (PKU) and hyperphenylalaninemia. SLC6A19-IN-1 is also suitable for studies related to various metabolic disorders, including tyrosinemia, maple syrup urine disease, urea cycle disorders, and hyperammonemia .

HY-P10563

Noraramtide BHV-1100	CD38	Cancer
Noraramtide (BHV-1100) is an antibody recruitment molecule. Noraramtide can specifically bind to CD38 molecules to recruit natural killer (NK) cells. Noraramtide enhances the ability of NK cells to kill tumor cells through antibody-dependent cellular cytotoxicity (ADCC). This mechanism allows NK cells to more effectively recognize and eliminate tumor cells while avoiding mutual killing between NK cells. Noraramtide can be used for the study of autologous cancer immunity .

Cat. No.	Product Name	Target	Research Area

HY-P10272

Rusfertide PTG-300	Ferroportin	Others
Rusfertide is a peptide mimetic of natural hepcidin, which targets and degrades ferroportin, reduces serum iron and transferrin-saturation, and thus regulates the production of red blood cells. Rusfertide ameliorates the polycythemia vera, β-thalassemia and hereditary hemochromatosis .

HY-P10563

Noraramtide BHV-1100	CD38	Cancer
Noraramtide (BHV-1100) is an antibody recruitment molecule. Noraramtide can specifically bind to CD38 molecules to recruit natural killer (NK) cells. Noraramtide enhances the ability of NK cells to kill tumor cells through antibody-dependent cellular cytotoxicity (ADCC). This mechanism allows NK cells to more effectively recognize and eliminate tumor cells while avoiding mutual killing between NK cells. Noraramtide can be used for the study of autologous cancer immunity .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-113371

2-Methylcitric acid Methylcitric acid	Human Gut Microbiota Metabolites Microorganisms Classification of Application Fields Ketones, Aldehydes, Acids Disease markers Endocrine diseases Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
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 .

HY-113371A

2-Methylcitric acid trisodium Methylcitric acid trisodium	Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
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 .

HY-113406

(±)-Hexanoylcarnitine DL-Hexanoylcarnitine	Natural Products Animals Classification of Application Fields Endogenous metabolite Inflammation/Immunology Disease Research Fields Source Classification	Others
(±)-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 .

Cat. No.	Product Name	Chemical Structure

HY-113371S

2-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 .

2-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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BODIPY 493/503 purchased from MedChemExpress. Usage Cited in: Nat Commun. 2025 Feb 1;16(1):1241. [Abstract]

MedchemExpress Validation 01

Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature. Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature. Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature.

BODIPY 493/503 purchased from MedChemExpress. Usage Cited in: Nat Commun. 2025 Feb 1;16(1):1241. [Abstract]

MedchemExpress Validation 01

MedchemExpress Validation 03

Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred

MedchemExpress Validation 04

MedchemExpress Validation

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