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inborn

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By Targets:	Endogenous Metabolite (14) Isotope-Labeled Compounds (2)
By Research Areas:	Cancer (2) Metabolic Disease (14) Neurological Disease (2)

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Isotope-Labeled Compounds

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-W017522

Adipic acid	Endogenous Metabolite	Metabolic Disease
Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism.

HY-113131A

Dihydroxyacetone phosphate hemimagnesium hydrate	Endogenous Metabolite	Metabolic Disease
Dihydroxyacetone phosphate hemimagnesium hydrate is an important intermediate in lipid biosynthesis and in glycolysis. It is a biochemical compound involved in many metabolic pathways, including the Calvin cycle in plants and glycolysis. Dihydroxyacetone phosphate hemimagnesium hydrate is found to be associated with transaldolase deficiency, which is an inborn error of metabolism .

HY-W017522S3

Adipic acid-13C	Endogenous Metabolite	Metabolic Disease
Adipic acid- ¹³C is the ¹³C labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-113131

Dihydroxyacetone phosphate	Endogenous Metabolite	Metabolic Disease
Dihydroxyacetone phosphate is an important intermediate in lipid biosynthesis and in glycolysis. It is a biochemical compound involved in many metabolic pathways, including the Calvin cycle in plants and glycolysis. Dihydroxyacetone phosphate is found to be associated with transaldolase deficiency, which is an inborn error of metabolism .

HY-W017522S6

Adipic acid-13C2	Isotope-Labeled Compounds	Metabolic Disease
Adipic acid- ¹³C₂ is ¹³C labeled Adipic acid. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism.

HY-W017522S

Adipic acid-d10	Endogenous Metabolite	Metabolic Disease
Adipic acid-d₁₀ is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S1

Adipic acid-13C6	Endogenous Metabolite	Metabolic Disease
Adipic acid- ¹³C₆ is the ¹³C labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S2

Adipic acid-d4	Endogenous Metabolite	Metabolic Disease
Adipic acid-d₄ is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S4

Adipic acid-d8	Endogenous Metabolite	Metabolic Disease
Adipic acid-d₈ is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S5

Adipic acid-d4-1	Endogenous Metabolite	Metabolic Disease
Adipic acid-d₄-1 is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-B0399

L-Carnitine 5+ Cited Publications (R)-Carnitine; Levocarnitine	Endogenous Metabolite	Neurological Disease Cancer
L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism .

HY-B2246

L-Carnitine hydrochloride 5+ Cited Publications (R)-Carnitine hydrochloride; Levocarnitine hydrochloride	Endogenous Metabolite	Metabolic Disease Cancer
L-Carnitine hydrochloride ((R)-Carnitine hydrochloride), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine hydrochloride functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine hydrochloride is an antioxidant. L-Carnitine hydrochloride can ameliorate metabolic imbalances in many inborn errors of metabolism .

HY-B0399S

L-Carnitine-d9 (R)-Carnitine-d₉; Levocarnitine-d₉	Isotope-Labeled Compounds Endogenous Metabolite	Neurological Disease Metabolic Disease
L-Carnitine-d₉ is the deuterium labeled L-Carnitine. L-Carnitine (Levocarnitine) is an endogenous molecule involved in fatty acid metabolism, biosynthesized within the human body using amino acids: L-lysine and L-methionine, as substrates. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2].

HY-B2246S

L-Carnitine-d9 chloride (R)-Carnitine-d₉ (chloride); Levocarnitine-d₉ (chloride)	Endogenous Metabolite	Metabolic Disease
L-Carnitine-d₉ (chloride)e is the deuterium labeled L-Carnitine chloride. L-Carnitine chloride, a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine chloride functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine chloride is an antioxidant. L-Carnitine chloride can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3].

HY-113410

3-Methylglutaric acid	Endogenous Metabolite	Metabolic Disease
3-Methylglutaric acid, a leucine metabolite, is a conspicuous C6 dicarboxylic organic acid classically associated with two distinct leucine pathway enzyme deficiencies, 3-hydroxy-3-methylglutaryl CoA lyase (HMGCL) and 3-methylglutaconyl CoA hydratase (AUH) .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-W017522

Adipic acid	Structural Classification Microorganisms Classification of Application Fields Ketones, Aldehydes, Acids Source classification Disease markers Metabolic Disease Endocrine diseases Endogenous metabolite Disease Research Fields Cardiovascular System Disorder Cancer	Endogenous Metabolite
Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism.

HY-113131A

Dihydroxyacetone phosphate hemimagnesium hydrate	Structural Classification Classification of Application Fields Ketones, Aldehydes, Acids Source classification Metabolic Disease Endogenous metabolite Disease Research Fields	Endogenous Metabolite
Dihydroxyacetone phosphate hemimagnesium hydrate is an important intermediate in lipid biosynthesis and in glycolysis. It is a biochemical compound involved in many metabolic pathways, including the Calvin cycle in plants and glycolysis. Dihydroxyacetone phosphate hemimagnesium hydrate is found to be associated with transaldolase deficiency, which is an inborn error of metabolism .

HY-B0399

L-Carnitine 5+ Cited Publications (R)-Carnitine; Levocarnitine	Structural Classification Natural Products Neurological Disease Source classification Endocrine diseases Endogenous metabolite Human Gut Microbiota Metabolites Microorganisms Immune System Disorder Disease markers Nervous System Disorder Disease Research Fields Cancer	Endogenous Metabolite
L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism .

HY-B2246

L-Carnitine hydrochloride 5+ Cited Publications (R)-Carnitine hydrochloride; Levocarnitine hydrochloride	Structural Classification Classification of Application Fields Ketones, Aldehydes, Acids Source classification Metabolic Disease Endogenous metabolite Disease Research Fields	Endogenous Metabolite
L-Carnitine hydrochloride ((R)-Carnitine hydrochloride), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine hydrochloride functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine hydrochloride is an antioxidant. L-Carnitine hydrochloride can ameliorate metabolic imbalances in many inborn errors of metabolism .

HY-113131

Dihydroxyacetone phosphate	Structural Classification Human Gut Microbiota Metabolites Microorganisms Classification of Application Fields Ketones, Aldehydes, Acids Source classification Metabolic Disease Endogenous metabolite Disease Research Fields	Endogenous Metabolite
Dihydroxyacetone phosphate is an important intermediate in lipid biosynthesis and in glycolysis. It is a biochemical compound involved in many metabolic pathways, including the Calvin cycle in plants and glycolysis. Dihydroxyacetone phosphate is found to be associated with transaldolase deficiency, which is an inborn error of metabolism .

HY-113410

3-Methylglutaric acid	Human Gut Microbiota Metabolites Microorganisms Classification of Application Fields Ketones, Aldehydes, Acids Source classification Disease markers Metabolic Disease Endocrine diseases Nervous System Disorder Endogenous metabolite Disease Research Fields Cardiovascular System Disorder	Endogenous Metabolite
3-Methylglutaric acid, a leucine metabolite, is a conspicuous C6 dicarboxylic organic acid classically associated with two distinct leucine pathway enzyme deficiencies, 3-hydroxy-3-methylglutaryl CoA lyase (HMGCL) and 3-methylglutaconyl CoA hydratase (AUH) .

Cat. No.	Product Name	Chemical Structure

HY-W017522S3

Adipic acid-13C
Adipic acid- ¹³C is the ¹³C labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S

Adipic acid-d10
Adipic acid-d₁₀ is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S2

Adipic acid-d4
Adipic acid-d₄ is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S6

Adipic acid-13C2
Adipic acid- ¹³C₂ is ¹³C labeled Adipic acid. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism.

HY-W017522S1

Adipic acid-13C6
Adipic acid- ¹³C₆ is the ¹³C labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S4

Adipic acid-d8
Adipic acid-d₈ is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-W017522S5

Adipic acid-d4-1
Adipic acid-d₄-1 is the deuterium labeled Adipic acid[1]. Adipic acid is found to be associated with HMG-CoA lyase deficiency, carnitine-acylcarnitine translocase deficiency, malonyl-Coa decarboxylase deficiency, and medium Chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism[2].

HY-B0399S

L-Carnitine-d9
L-Carnitine-d₉ is the deuterium labeled L-Carnitine. L-Carnitine (Levocarnitine) is an endogenous molecule involved in fatty acid metabolism, biosynthesized within the human body using amino acids: L-lysine and L-methionine, as substrates. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2].

L-Carnitine-d9

L-Carnitine-d₉ is the deuterium labeled L-Carnitine. L-Carnitine (Levocarnitine) is an endogenous molecule involved in fatty acid metabolism, biosynthesized within the human body using amino acids: L-lysine and L-methionine, as substrates. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2].

HY-B2246S

L-Carnitine-d9 chloride
L-Carnitine-d₉ (chloride)e is the deuterium labeled L-Carnitine chloride. L-Carnitine chloride, a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine chloride functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine chloride is an antioxidant. L-Carnitine chloride can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3].

L-Carnitine-d9 chloride

L-Carnitine-d₉ (chloride)e is the deuterium labeled L-Carnitine chloride. L-Carnitine chloride, a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine chloride functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine chloride is an antioxidant. L-Carnitine chloride can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3].

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inborn

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Isotope-Labeled Compounds

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