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

Amino Acid/Protein Metabolism

Amino acid or protein metabolism disorders with epilepsy involve inherited metabolic defects that disrupt the normal breakdown or synthesis of amino acids or proteins, leading to the accumulation of toxic metabolites or deficiencies in essential compounds. These conditions often present with neurological symptoms, including seizures (epilepsy), developmental delay, intellectual disability, and abnormal muscle tone. Common examples include urea cycle disorders (e.g., ornithine transcarbamylase deficiency), organic acidemias (e.g., methylmalonic acidemia), and aminoacidopathies (e.g., phenylketonuria, maple syrup urine disease). Early diagnosis through newborn screening and prompt treatment—such as dietary restriction of specific amino acids, supplementation with cofactors or alternative pathways, and management of metabolic crises—are critical to preventing severe neurological damage and improving outcomes. Epilepsy in these disorders may result from neurotoxic metabolite buildup, energy deficiency in neurons, or disruption of neurotransmitter balance.

 

Amino Acid/Protein Metabolism (18):

Cat. No. 상품명 CAS No. Purity 화학구조
  • 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-W015590
    2-Hydroxyphenylacetic acid 614-75-5 98.79%
    2-Hydroxyphenylacetic acid is a metabolite of Phenylalanine and Tyrosine, as well as a product of the biotransformation of Phenylacetonitrile by marine fungi. 2-Hydroxyphenylacetic acid acts as a potential biomarker for food. 2-Hydroxyphenylacetic acid slightly induces the expression of green fluorescent protein. 2-Hydroxyphenylacetic acid is used in the research of phenylketonuria and related hyperphenylalaninemia.
    2-Hydroxyphenylacetic acid
  • 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
  • HY-13495
    ML281 1404437-62-2 98.04%
    ML281 is a highly selective inhibitor of serine/threonine kinase 33 (STK33) with an IC50 value of 14 nM. ML281 shows 700-fold selectivity over PKA and 550-fold over AurB. ML281 exerts core mechanism by inhibiting STK33: in small cell lung cancer, ML281 downregulates RPS6/BAD signaling phosphorylation, induces apoptosis, and suppresses proliferation, invasion. ML281 reduces STK33-mediated 4-hydroxyphenylpyruvate dioxygenase (HPD) phosphorylation in tyrosinemia . ML281 is suitable for research on STK33 function, KRAS mutation-related cancers (pancreatic cancer, colon cancer, lung adenocarcinoma, etc.), small cell lung cancer, and tyrosinemia-related damage
    ML281
  • HY-B1713A
    DL-Ornithine hydrochloride 1069-31-4 99.78%
    DL-Ornithine (DL-(±)-Ornithine) hydrochloride is an amino acid and also a urea cycle promoter. DL-Ornithine hydrochloride has the characteristics of low metabolic rate and rapid excretion, and only the L-enantiomer undergoes decarboxylation. In mice inoculated with L-1210 leukemia cells, DL-Ornithine hydrochloride is excreted in urine in its original form, and its conversion to carbon dioxide within 24 h is negligible. DL-Ornithine hydrochloride is mainly used in the urea cycle to eliminate excess nitrogen in the body and protect the kidneys.
    DL-Ornithine hydrochloride
  • HY-B1713
    Ornithine 616-07-9
    Ornithine ((±)-Ornithine) is a non-essential amino acid. Ornithine is an intermediate in urea cycle and acts as a substrate for these rate limiting enzymes ornithine transcarbomylase, ornithine aminotransferase and ornithine decarboxylase producing citrulline, proline and polyamines, respectively. Ornithine can be used in the research of hyperornithinemia, hyperammonemia, and cancer.
    Ornithine
  • HY-179581
    SLC6A19-IN-4 2962069-39-0 99.55%
    SLC6A19-IN-4 is an allosteric-competitive and orally active B0AT1 inhibitor. SLC6A19-IN-4 inhibits both human and mouse B0AT1 with IC50 values of 513 nM and 295 nM, respectively. SLC6A19-IN-4 exhibits excellent metabolic stability. SLC6A19-IN-4 significantly increases urinary phenylalanine (Phe) excretion and reduces plasma Phe levels through dual inhibition of B0AT1 in both the intestine (reducing absorption) and kidney (promoting excretion) in vivo. SLC6A19-IN-4 can be used for phenylketonuria (PKU) and other disorders involving SLC6-family transporters research.
    SLC6A19-IN-4
  • HY-175151
    2-Amino-3-[2-(tert-butoxy)-2-oxoethoxy]propanoic acid
    2-Amino-3-[2-(tert-butoxy)-2-oxoethoxy]propanoic acid (O-(2-(tert-Butoxy)-2-oxoethyl)serine) is a Serine (HY-N0650) derivative. Serine is an amino acid.
    2-Amino-3-[2-(tert-butoxy)-2-oxoethoxy]propanoic acid
  • HY-134421
    2-Butenoyl coenzyme A lithium 102680-35-3 98%
    2-Butenoyl coenzyme A lithium is an inactivator and a substrate of Plasmodium falciparum enoyl-β-hydroxyacyl-acyl carrier protein (ACP) reductase and other enoyl-CoA reductases, and it is also the lithium salt of trans-2-methyl-2-butenoyl coenzyme A. 2-Butenoyl coenzyme A lithium acts on short-chain and medium-chain coenzyme A dehydrogenases as well as glutaryl-CoA dehydrogenase, and shows no activity against wild-type isovaleryl-CoA dehydrogenase. 2-Butenoyl coenzyme A lithium functions as a metabolite in the L-isoleucine catabolic pathway, and can serve as a substrate in the activity assay of 3-ketothiolase. 2-Butenoyl coenzyme A lithium is applicable to research related to 3-ketothiolase deficiency.
    2-Butenoyl coenzyme A lithium
  • HY-P990952
    Zaltenibart 2527079-60-1 99%
    Zaltenibart (OMS906) is an IgG4 humanized monoclonal antibody and MASP-3 inhibitor. By inhibiting MASP-3, Zaltenibart achieves upstream inhibition of the alternative complement pathway. MASP-3 is an upstream activator of Factor D (Factor D), a key enzyme in the alternative complement pathway. Zaltenibart prevents both intravascular hemolysis and extravascular hemolysis. Zaltenibart is applicable to research related to paroxysmal nocturnal hemoglobinuria.
    Zaltenibart
  • HY-135484
    NDSB-195 160255-06-1 99.93%
    NDSB-195 is protein stabilizer that enhances the dynamics of the β4-α2 loop of ubiquitin. NDSB-195 stabilizes Saccharomyces cerevisiae ubiquitin against guanidium chloride denaturation, increasing the onset temperature of heat denaturation in the presence of the denaturant.
    NDSB-195
  • HY-131516
    Tiopronin-cysteine disulfide 77591-18-5
    Tiopronin-cysteine disulfide is a soluble disulfide metabolite/derivative of Tiopronin (HY-B0373) formed by reaction of Tiopronin with cystine. Tiopronin-cysteine disulfide reduces cystine crystal formation in cystinuria. Tiopronin-cysteine disulfide can be used for the research of cystinuria.
    Tiopronin-cysteine disulfide
  • HY-W040088
    L-Alanyl-L-leucine 3303-34-2 98.15%
    L-Alanyl-L-leucine is a competitive inhibitor of small intestinal glycyl-L-leucine hydrolase with Ki values of 0.53 mM (phosphate buffer) or 0.22 mM (Tris buffer). L-Alanyl-L-leucine can be used for research on Hartnup disease and cystinuria.
    L-Alanyl-L-leucine
  • HY-W040088R
    L-Alanyl-L-leucine (Standard) 3303-34-2
    L-Alanyl-L-leucine is an endogenous metabolite. This product is used for research and analytical applications. L-Alanyl-L-leucine is a competitive inhibitor of small intestinal glycyl-L-leucine hydrolase with Ki values of 0.53 mM (phosphate buffer) or 0.22 mM (Tris buffer). L-Alanyl-L-leucine can be used for research on Hartnup disease and cystinuria.
    L-Alanyl-L-leucine (Standard)
  • HY-179107
    SLC6A19-IN-3 3104477-57-5
    SLC6A19-IN-3 (Compound 83-P1-P2) is a potent, selective and orally active SLC6A19 inhibitor with an IC50 of 28 nM. SLC6A19-IN-3 can block SLC6A19-mediated transmembrane transport of phenylalanine, reducing intestinal absorption of phenylalanine from food and renal tubular reabsorption of phenylalanine. SLC6A19-IN-3 can be used for the research of metabolic disease, such as phenylketonuria.
    SLC6A19-IN-3
  • HY-158161
    SLC6A19-IN-1 3032920-98-9
    SLC6A19-IN-1 is a potent and highly selective inhibitor and transport corrector of human SLC6A19 (IC50=47 nM). Furthermore, at a concentration of 35 μM, SLC6A19-IN-1 exhibits no activity against SLC1A5, SLC7A5, or SLC6A8. SLC6A19-IN-1 is applicable to research on phenylketonuria (PKU) and hyperphenylalaninemia. SLC6A19-IN-1 is also suitable for studies related to various metabolic disorders, including tyrosinemia, maple syrup urine disease, urea cycle disorders, and hyperammonemia.
    SLC6A19-IN-1
  • HY-P2951
    Phenylalanine ammonia-lyase 9024-28-6
    Phenylalanine ammonia-lyase is a non-mammalian enzyme converting phenylalanine into trans-cinnamic acid and ammonia. Phenylalanine ammonia-lyase can be used in the research of phenylketonuria.
    Phenylalanine ammonia-lyase
  • HY-181619
    TAO Kinase-IN-3 3114565-22-6
    TAO Kinase-IN-3 is a potent pan-TAOK (Thousand-And-One Kinase) inhibitor that specifically binds to and inhibits the kinase activities of TAOK1, TAOK2 and TAOK3 with Kd values of <0.17 nM, 0.34 nM, and 0.17 nM, respectively. TAO Kinase-IN-3 can be used to study Alzheimer's disease, primary tauopathies and related syndromes. In addition, TAO Kinase-IN-3 is also widely used in research on the mechanisms of cancer cachexia and the muscle atrophy it induces.
    TAO Kinase-IN-3