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inborn

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By Targets:	Drug Metabolite (2) Endogenous Metabolite (20) Isotope-Labeled Compounds (3)
By Research Areas:	Cancer (5) Cardiovascular Disease (1) Metabolic Disease (17) Neurological Disease (4)

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Natural
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Isotope-Labeled Compounds

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-W017522

Adipic acid	Drug Metabolite 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-W017522R

Adipic acid (Standard)	Drug Metabolite Endogenous Metabolite	Metabolic Disease
Adipic acid (Standard) is the analytical standard of Adipic acid. This product is intended for research and analytical applications. 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-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-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-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-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-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-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-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 Maximum Cited Publications 16 Publications Verification (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 Maximum Cited Publications 16 Publications Verification (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-B2246S

L-Carnitine-d9 chloride (R)-Carnitine-d₉ chloride; Levocarnitine-d₉ chloride	Isotope-Labeled Compounds 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-B0399G

L-Carnitine (R)-Carnitine; Levocarnitine	Endogenous Metabolite	Neurological Disease Cancer
L-Carnitine (GMP) is L-Carnitine (HY-B0399) produced by using GMP guidelines. L-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-B0399R

L-Carnitine (Standard)	Endogenous Metabolite	Neurological Disease Cancer
L-Carnitine (Standard) is the analytical standard of L-Carnitine. This product is intended for research and analytical applications. 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-B2246R

L-Carnitine (hydrochloride) (Standard)	Endogenous Metabolite	Metabolic Disease Cancer
L-Carnitine (hydrochloride) (Standard) is the analytical standard of L-Carnitine (hydrochloride). This product is intended for research and analytical applications. 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 1 Publications Verification (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-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) .

HY-113410R

3-Methylglutaric acid (Standard)	Endogenous Metabolite	Metabolic Disease

HY-W741510

21-Deoxy Cortisone 21-Desoxycortisone; NSC 38722	Endogenous Metabolite	Cardiovascular Disease
21-Deoxy Cortisone (21-Desoxycortisone; NSC 38722) is a corticosteroid metabolite of 11-ketoprogesterone. It is formed from 11-ketoprogesterone by the cytochrome P450 (CYP) isozyme CYP17A1, but can also be produced by oxidation of 21-deoxycortisone (HY-113405) by 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2). Congenital adrenal hyperplasia is an inborn error of metabolism characterized by a deficiency of 21-hydroxylase, and 21-Deoxy Cortisone levels are elevated in patients with congenital adrenal hyperplasia.

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 Endocrine diseases Metabolic Disease Endogenous metabolite Disease Research Fields Cardiovascular System Disorder Cancer	Drug Metabolite 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-W017522R

Adipic acid (Standard)	Structural Classification Microorganisms Ketones, Aldehydes, Acids Source classification Disease markers Endocrine diseases Endogenous metabolite Cardiovascular System Disorder Cancer	Drug Metabolite Endogenous Metabolite
Adipic acid (Standard) is the analytical standard of Adipic acid. This product is intended for research and analytical applications. 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-B0399

L-Carnitine 15+ Cited Publications (R)-Carnitine; Levocarnitine	Structural Classification Natural Products Neurological Disease Source classification Endocrine diseases Endogenous metabolite Human Gut Microbiota Metabolites Immune System Disorder Microorganisms 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 15+ 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-B0399R

L-Carnitine (Standard)	Structural Classification Natural Products Human Gut Microbiota Metabolites Immune System Disorder Microorganisms Source classification Disease markers Endocrine diseases Nervous System Disorder Endogenous metabolite Cancer	Endogenous Metabolite
L-Carnitine (Standard) is the analytical standard of L-Carnitine. This product is intended for research and analytical applications. 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-113410

3-Methylglutaric acid	Structural Classification Classification of Application Fields Source classification Endocrine diseases Metabolic Disease Endogenous metabolite Human Gut Microbiota Metabolites Microorganisms Ketones, Aldehydes, Acids Disease markers Nervous System Disorder 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) .

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-B2246R

L-Carnitine (hydrochloride) (Standard)	Structural Classification Ketones, Aldehydes, Acids Source classification Endogenous metabolite	Endogenous Metabolite
L-Carnitine (hydrochloride) (Standard) is the analytical standard of L-Carnitine (hydrochloride). This product is intended for research and analytical applications. 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-113410R

3-Methylglutaric acid (Standard)	Structural Classification Human Gut Microbiota Metabolites Microorganisms Ketones, Aldehydes, Acids Source classification Disease markers Endocrine diseases Nervous System Disorder Endogenous metabolite Cardiovascular System Disorder	Endogenous Metabolite

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-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-B0399S

L-Carnitine-d9 1 Publications Verification
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

1 Publications Verification

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-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-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-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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