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primary hyperoxaluria type 2

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Endogenous Metabolite (5) Mitochondrial Metabolism (1)
By Research Areas:	Cancer (1) Inflammation/Immunology (1) Metabolic Disease (6) Neurological Disease (1)
Click Chemistry:	Alkynes (1)

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Click Chemistry

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-142035

N-Propargylglycine 1 Publications Verification	Mitochondrial Metabolism	Neurological Disease Metabolic Disease Inflammation/Immunology Cancer
N-Propargylglycine is a brain-penetrant and orally active PRODH inhibitor. N-Propargylglycine covalently modifies enzyme-bound FAD and active site lysine, causing enzyme structural distortion, protein decay, and irreversible inhibition of proline and 4-hydroxyproline catabolism. N-Propargylglycine induces UPRmt, upregulates mitochondrial chaperones and YME1L1, enhances mitochondrial proteostasis, blocks astrocytic L-proline consumption, and abolishes L-proline’s ATP-maintaining and viability-protective effects. N-Propargylglycine stimulates neural processes, increases brain proline, hydroxyproline, and sarcosine levels, partially normalizes Huntington’s disease whole brain transcriptomes. N-Propargylglycine reduces hyperoxaluria, prevents calcium oxalate stone formation, reduces kidney tubular damage, and restores weight and survival in Grhpr knockout mice. N-Propargylglycine can be used for the research of breast cancer, neurodegenerative disorders, Huntington’s disease, and primary hyperoxaluria type 2 .

HY-139070A

D-Glyceric acid sodium	Endogenous Metabolite	Metabolic Disease
D-Glyceric acid sodium is the sodium form of D-Glyceric acid (HY-139070A). D-Glyceric acid is an endogenous metabolite present in urine that can be used for the research of primary hyperoxaluria type I and glutaric acidemia type 2 .

HY-113377

L-Glyceric acid	Endogenous Metabolite	Metabolic Disease
L-Glyceric acid is a mainly urinary metabolite accumulating in rare inherited metabolic disease L-glyceric aciduria. L-Glyceric acid can be used to diagnose primary hyperoxaluria type 2 (PH2). L-Glyceric acid excretion to distinguish PH1 from PH2 .

HY-139070

D-Glyceric acid	Endogenous Metabolite	Metabolic Disease
D-Glyceric acid is an endogenous metabolite present in urine that can be used for the research of primary hyperoxaluria type I and glutaric acidemia type 2 .

HY-113377A

L-Glyceric acid sodium	Endogenous Metabolite	Metabolic Disease
L-Glyceric acid sodium is a mainly urinary metabolite accumulating in rare inherited metabolic disease L-glyceric aciduria. L-Glyceric acid sodium can be used to diagnose primary hyperoxaluria type 2 (PH2). L-Glyceric acid sodium excretion to distinguish PH1 from PH2 .

HY-W584173

D-Glyceric acid calcium hydrate	Endogenous Metabolite	Metabolic Disease
D-Glyceric acid calcium hydrate is an endogenous metabolite present in urine that can be used for the research of primary hyperoxaluria type I and glutaric acidemia type 2 .

D-Glyceric acid calcium hydrate	Biochemical Assay Reagents
D-Glyceric acid calcium hydrate is an endogenous metabolite present in urine that can be used for the research of primary hyperoxaluria type I and glutaric acidemia type 2 .

L-Glyceric acid	Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
L-Glyceric acid is a mainly urinary metabolite accumulating in rare inherited metabolic disease L-glyceric aciduria. L-Glyceric acid can be used to diagnose primary hyperoxaluria type 2 (PH2). L-Glyceric acid excretion to distinguish PH1 from PH2 .

D-Glyceric acid	Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
D-Glyceric acid is an endogenous metabolite present in urine that can be used for the research of primary hyperoxaluria type I and glutaric acidemia type 2 .

L-Glyceric acid sodium	Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
L-Glyceric acid sodium is a mainly urinary metabolite accumulating in rare inherited metabolic disease L-glyceric aciduria. L-Glyceric acid sodium can be used to diagnose primary hyperoxaluria type 2 (PH2). L-Glyceric acid sodium excretion to distinguish PH1 from PH2 .

Cat. No.	Product Name		Classification

HY-142035

N-Propargylglycine 1 Publications Verification		Alkynes
N-Propargylglycine is a brain-penetrant and orally active PRODH inhibitor. N-Propargylglycine covalently modifies enzyme-bound FAD and active site lysine, causing enzyme structural distortion, protein decay, and irreversible inhibition of proline and 4-hydroxyproline catabolism. N-Propargylglycine induces UPRmt, upregulates mitochondrial chaperones and YME1L1, enhances mitochondrial proteostasis, blocks astrocytic L-proline consumption, and abolishes L-proline’s ATP-maintaining and viability-protective effects. N-Propargylglycine stimulates neural processes, increases brain proline, hydroxyproline, and sarcosine levels, partially normalizes Huntington’s disease whole brain transcriptomes. N-Propargylglycine reduces hyperoxaluria, prevents calcium oxalate stone formation, reduces kidney tubular damage, and restores weight and survival in Grhpr knockout mice. N-Propargylglycine can be used for the research of breast cancer, neurodegenerative disorders, Huntington’s disease, and primary hyperoxaluria type 2 .

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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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