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glutaric aciduria type I

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Endogenous Metabolite (11) Isotope-Labeled Compounds (2) MOFs (1) Reference Standards (3)
By Research Areas:	Metabolic Disease (11)

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Natural
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Isotope-Labeled Compounds

Targets Recommended: C-type Lectin-like Receptors (CTLRs) PCSK9 Scavenger Receptor Class B type I (SR-BI）

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-113005

Glutarylcarnitine	Endogenous Metabolite	Metabolic Disease
Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.

HY-W008820

Glutaric acid	MOFs Endogenous Metabolite	Metabolic Disease
Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-W008820S

Glutaric acid-d6 1 Publications Verification	Endogenous Metabolite	Metabolic Disease
Glutaric acid-d₆ is the deuterium labeled Glutaric acid. Glutaric acid is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-CE02028

Glutaconyl-CoA Glutaconyl-coenzyme A	Endogenous Metabolite	Metabolic Disease
Glutaconyl-CoA (Glutaconyl-coenzyme A) is a key metabolic intermediate in the mitochondrial catabolic pathways of lysine, hydroxylysine and tryptophan. Glutaconyl-CoA competitively inhibits wild-type glutaryl-CoA dehydrogenase (GCDH) with a K_i value of 1.1 µM. Glutaconyl-CoA is generated via the oxidative decarboxylation of glutaryl-CoA catalyzed by GCDH, and is subsequently further metabolized. Glutaconyl-CoA can be used in the research of type I glutaric aciduria .

HY-113005A

Glutarylcarnitine lithium	Endogenous Metabolite	Metabolic Disease
Glutarylcarnitine lithium is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes .

HY-W008820R

Glutaric acid (Standard)	Reference Standards Endogenous Metabolite	Metabolic Disease
Glutaric acid (Standard) is the analytical standard of Glutaric acid. This product is intended for research and analytical applications. Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I . In Vitro: Glutaric acid (GA) at concentrations of 1 and 2 mM is able to reduce TRAP measurement by up to 28% in a dose-dependent manner (β=0.77; P<0.001). Furthermore, a significantly inverse correlation is also verified between chemiluminescence and TRAP (β=0.81; P<0.001). Glutaric acid does not alter the activities of Cat and SOD, but strongly inhibits (up to 46%) the activity of GPx even at the lower concentration used (0.5 mM). It is observed that the metabolite inhibits this activity in a dose-dependent manner at concentrations as low as 0.05 mM .

HY-W008820S1

Glutaric acid-d4	Endogenous Metabolite	Metabolic Disease
Glutaric acid-d₄ is the deuterium labeled Glutaric acid. Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-113005S

Glutarylcarnitine-d9 chloride	Isotope-Labeled Compounds Endogenous Metabolite	Metabolic Disease
Glutarylcarnitine-d₉ (chloride) is the deuterium labeled Glutarylcarnitine chloride. Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.

HY-113005R

Glutarylcarnitine (Standard)	Endogenous Metabolite Reference Standards	Metabolic Disease
Glutarylcarnitine (Standard) is the analytical standard of Glutarylcarnitine. This product is intended for research and analytical applications. Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.

HY-W008820S2

Glutaric acid-d2	Isotope-Labeled Compounds Endogenous Metabolite	Metabolic Disease
Glutaric acid-d₂ is the deuterium labeled Glutaric acid. Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-113005AR

Glutarylcarnitine lithium (Standard)	Endogenous Metabolite Reference Standards	Metabolic Disease
Glutarylcarnitine (lithium) (Standard) is the analytical standard of Glutarylcarnitine (lithium). This product is intended for research and analytical applications. Glutarylcarnitine lithium is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-113005

Glutarylcarnitine	Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.

HY-W008820

Glutaric acid	Structural Classification Microorganisms Classification of Application Fields Ketones, Aldehydes, Acids Disease markers Endocrine diseases Metabolic Disease Nervous System Disorder Endogenous metabolite Disease Research Fields Source Classification	MOFs Endogenous Metabolite
Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-113005A

Glutarylcarnitine lithium	Human Gut Microbiota Metabolites Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
Glutarylcarnitine lithium is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes .

HY-W008820R

Glutaric acid (Standard)	Structural Classification Microorganisms Ketones, Aldehydes, Acids Disease markers Endocrine diseases Nervous System Disorder Endogenous metabolite Source Classification	Reference Standards Endogenous Metabolite
Glutaric acid (Standard) is the analytical standard of Glutaric acid. This product is intended for research and analytical applications. Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I . In Vitro: Glutaric acid (GA) at concentrations of 1 and 2 mM is able to reduce TRAP measurement by up to 28% in a dose-dependent manner (β=0.77; P<0.001). Furthermore, a significantly inverse correlation is also verified between chemiluminescence and TRAP (β=0.81; P<0.001). Glutaric acid does not alter the activities of Cat and SOD, but strongly inhibits (up to 46%) the activity of GPx even at the lower concentration used (0.5 mM). It is observed that the metabolite inhibits this activity in a dose-dependent manner at concentrations as low as 0.05 mM .

HY-113005R

Glutarylcarnitine (Standard)	Structural Classification Ketones, Aldehydes, Acids Endogenous metabolite Source Classification	Endogenous Metabolite Reference Standards
Glutarylcarnitine (Standard) is the analytical standard of Glutarylcarnitine. This product is intended for research and analytical applications. Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.

HY-113005AR

Glutarylcarnitine lithium (Standard)	Structural Classification Human Gut Microbiota Metabolites Ketones, Aldehydes, Acids Endogenous metabolite Source Classification	Endogenous Metabolite Reference Standards
Glutarylcarnitine (lithium) (Standard) is the analytical standard of Glutarylcarnitine (lithium). This product is intended for research and analytical applications. Glutarylcarnitine lithium is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes .

Cat. No.	Product Name	Chemical Structure

HY-W008820S

Glutaric acid-d6 1 Publications Verification
Glutaric acid-d₆ is the deuterium labeled Glutaric acid. Glutaric acid is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-W008820S1

Glutaric acid-d4
Glutaric acid-d₄ is the deuterium labeled Glutaric acid. Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

HY-113005S

Glutarylcarnitine-d9 chloride
Glutarylcarnitine-d₉ (chloride) is the deuterium labeled Glutarylcarnitine chloride. Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.

HY-W008820S2

Glutaric acid-d2
Glutaric acid-d₂ is the deuterium labeled Glutaric acid. Glutaric acid, C5 dicarboxylic acid, is an intermediate during the catabolic pathways of lysine and tryptophan. Glutaric acid affects pericyte contractility and migration. Glutaric acid is an indicator of glutaric aciduria type I .

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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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glutaric aciduria type I

Inhibitors & Agonists

NaturalProducts

Isotope-Labeled Compounds

Natural
Products