2. Disease Areas
  3. Metabolic or Endocrine Disease
  4. Amino Acid/Protein Metabolism
  5. Organic Aciduria

Organic Aciduria

Organic aciduria refers to a group of metabolic disorders characterized by the abnormal accumulation of organic acids in the body due to defects in amino acid metabolism, particularly involving branched-chain amino acids, resulting in clinical manifestations ranging from acute neurological symptoms to chronic conditions such as dementia.

Organic Aciduria (2):

Cat. No. Product Name CAS No. Purity Chemical Structure
  • HY-142035
    N-Propargylglycine 58160-95-5 99.70%
    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.
    N-Propargylglycine
  • HY-W019724
    2,2-Dihydroxyacetic acid 563-96-2 98.0%
    2,2-Dihydroxyacetic acid is an endogenous metabolite, which is the monohydrate of Glyoxylic Acid. 2,2-Dihydroxyacetic acid may participate in the microbial glyoxylate cycle, induce an increase in reactive oxygen species, promote cell differentiation, and modify proteins to form advanced glycation end products (AGEs) (HY-NP165). 2,2-Dihydroxyacetic acid is associated with metabolic diseases such as primary hyperoxaluria.
    2,2-Dihydroxyacetic acid