95
53
Cat. No. | Product Name | Target | Signaling Pathways |
---|---|---|---|
T12499 |
PKR-IN-2
|
PKM | Metabolism |
PKR-IN-2 是丙酮酸激酶PKR 亚型的激活剂,可用于 PKR 功能相关疾病的研究,包括癌症、糖尿病、肥胖、自身免疫性疾病、良性前列腺增生等。 | |||
T4223 |
Mitapivat
PKR-IN-1 |
PKM | Metabolism |
Mitapivat (PKR-IN-1) 是口服具有活性的变构丙酮酸激酶 (pyruvate kinase) 激活剂,其AC50=20 nM。它在广泛的 PKLR 基因型中增加酶活性、蛋白质稳定性和 ATP 水平。它有潜在的恢复 PK 缺乏的糖酵解途径活性的作用。 | |||
T41160 |
Lalistat 2
|
Lipase | Metabolism |
Lalistat 2 是一种溶酶体酸性脂肪酶的特异性抑制剂,IC50 为 152 nM,对人胰脂肪酶或牛乳脂蛋白脂肪酶没有抑制作用(高达 10 μM)。 | |||
T1536 |
Promestriene
3-propyl ethyl,舒经劳,17B-methyl estradiol,Promestriano |
Estrogen/progestogen Receptor | Endocrinology/Hormones |
Promestriene (3-propyl ethyl) 是局部有效的雌激素,是合成的雌二醇二乙醚。它对阴道萎缩有作用,但吸收量小。 | |||
T29856 |
Aleplasinin
PAZ 417,PAZ417,PAZ-417 |
PAI-1 | Metabolism |
Aleplasinin (PAZ 417) 是纤溶酶原激活物抑制剂 1 (PAI-1) 的选择性和口服活性抑制剂,是纤溶系统的关键负调节剂。 Aleplasinin 可用于阿尔茨海默病研究。 | |||
T4505 |
Ferrous Bisglycinate
Bisglycine iron(II) salt,Iron(II) bisglycinate,甘氨酸亚铁 |
Estrogen/progestogen Receptor | Endocrinology/Hormones |
Ferrous Bisglycinate (Bisglycine iron(II) salt) 是口服具有活力的铁强化剂剂补充剂。它可用于研究缺铁性贫血。 | |||
T13692 |
Ferric maltol
Iron(III) 2-methyl-4-oxo-4H-pyran-3-olate,麦芽酚铁 |
Others | Others |
Ferric maltol (Iron(III) 2-methyl-4-oxo-4H-pyran-3-olate) 是一种具有口服活性的单一铁离子 (Fe3+) 复合物,可用于研究炎症性肠病缺铁性贫血。 | |||
T7313 |
Enclomiphene citrate
恩氯米芬柠檬酸盐,Enclomifene citrate,(E)-Clomiphene citrate,trans-Clomiphene citrate |
Estrogen Receptor/ERR | Endocrinology/Hormones |
Enclomiphene citrate (trans-Clomiphene citrate) 是口服具有活力的 oestrogen receptor 拮抗剂,具有抗雌激素活性。 | |||
T13396 |
Zinc Protoporphyrin
Zinc Protoporphyrin-9,锌原卟啉,ZnPP,Zn(II)-protoporphyrin IX |
Apoptosis; Reactive Oxygen Species; Endogenous Metabolite | Apoptosis; Immunology/Inflammation; Metabolism; NF-κB |
Zinc Protoporphyrin (Zn(II)-protoporphyrin IX) 是一种具有口服活性的,竞争性血红素加氧酶-1 抑制剂,显著减弱间苯三酚对 H2O2 的保护作用,具有抗癌活性。它用于评估人群铁状态与血红蛋白浓度的组合,也可用作个体孕妇和儿童缺铁的筛查标志物。 | |||
T8132 |
Iron sucrose
Iron saccharate,蔗糖铁,Sucroferric oxyhydroxide |
Reactive Oxygen Species | Immunology/Inflammation; Metabolism; NF-κB |
Iron sucrose (Sucroferric oxyhydroxide) 是一种静脉注射铁制剂,也是一种促氧化剂,可用于研究缺铁性贫血。 | |||
T3036 |
Dydrogesterone
|
Progesterone Receptor | Others |
Dydrogesterone 是一种口服有效的孕激素,可作用于孕酮缺乏相关疾病。 | |||
T20093 |
p-Phenylenediamine, N,N'-diphenyl-
NSC 761,NSC 5761,Flexamine G,NSC-5761,N,N'-二苯基-1,4-苯二胺,DPPD |
Others | Others |
p-Phenylenediamine, N,N'-diphenyl- (NSC-761) 是一种生物活性化学品。它已被用于预防羔羊维生素 E 缺乏症。 | |||
T4994 |
Tocofersolan
Vitamin E-TPGS,Tocophersolan,TPGS,D-α-Tocopherol polyethylene glycol 1000 succinate,托可索仑,维生素E聚乙二醇琥珀酸酯 |
Others | Others |
Tocofersolan (TPGS) 是合成的 α-生育酚的聚乙二醇衍生物。 | |||
T61144 |
Hepcidin antagonist-1
|
Others | Others |
Hepcidin antagonist-1 是一种铁调素拮抗剂。Hepcidin antagonist-1 可用于研究像缺铁疾病和贫血类的代谢紊乱。 | |||
T4552 |
Fursultiamine
Diteftin,呋喃硫胺,Judolor,Alinamin F |
Others | Others |
Fursultiamine (Alinamin F) 是维生素 B1衍生物,具有抗伤害和抗肿瘤作用。它可用于研究维生素 B1缺乏病、骨关节炎和癌症。 | |||
T17176 |
Salicyl-AMS
|
Antibacterial | Microbiology/Virology |
Salicyl-AMS 是一种分支杆菌生长素生物合成抑制剂,能在体外缺铁的情况下抑制分枝杆菌的生长。 | |||
T5963 |
Menadione bisulfite sodium
Sodium menadione bisulfite,亚硫酸氢钠甲萘醌,Menadione sodium bisulfite,Vitamin K3 sodium bisulfite |
Others | Others |
Menadione bisulfite sodium (Vitamin K3 sodium bisulfite) 是可以诱导急性氧化应激的试剂,可用作植物防御活化剂,具有抵抗病菌的作用。 | |||
T20506 |
Maleuric acid
N-Carbamoylmaleamic acid,AI3 32778,AI3-32778,AI332778 |
Others | Others |
Maleuric acid (N-Carbamoylmaleamic acid) 可抗有丝分裂,可与放射自显影技术结合起来用于生物研究。Maleuric acid 可用于改善动植物的生长和繁殖力以及治疗乳清酸缺乏症。 | |||
T10393 |
AT-007
|
Reductase | Endocrinology/Hormones; Metabolism |
AT-007 是口服有效的,中枢神经系统渗透性醛糖还原酶的高效抑制剂, IC50为 100 pM。在 GALT 缺失大鼠中,它降低了有毒的半乳糖醇水平并预防了疾病并发症。 | |||
T13219 |
TTA-Q6
|
Calcium Channel | Membrane transporter/Ion channel; Metabolism |
TTA-Q6 是一种选择性 T 型 Ca2+ 通道拮抗剂,具有潜在的抗肿瘤和免疫调节活性,可抑制肿瘤细胞摄取细胞外钙离子,诱导细胞内钙缺乏和内质网(ER)应激,用于治疗神经系统疾病。 | |||
T13730 |
Hydroxocobalamin acetate
|
Others | Others |
Hydroxocobalamin acetate 是可注射的天然维生素 B12,其不良反应特征良好,可用于研究维生素 B12 缺乏症 (包括恶性贫血) 。 | |||
T37192 |
4-Methylumbelliferyl-α-D-Galactopyranoside
4MU-α-Gal |
Others | Others |
4-Methylumbelliferyl-α-D-Galactopyranoside (4MU-α-Gal) (4-MU-α-Gal)是α-半乳糖苷酶的荧光底物。除了用于表征新的α-半乳糖苷酶外,4-MU-α-Gal 还用于评估与法布里病相关的α-半乳糖糖苷酶活性的缺乏。 | |||
T4512 |
Magnesium glycinate
magnesium 2-aminoacetate,Magnesium diglycinate,甘氨酸镁 |
Others | Others |
Magnesium glycinate (magnesium 2-aminoacetate) 是一种营养补充剂,是甘氨酸的镁盐,具有良好的理化性质和生物活性。Magnesium 是人体必需的矿物质,参与能量代谢过程,协助维持正常的肌肉功能,对人体起着至关重要的作用。 | |||
T77599 |
Methyl retinoate
Retinoic acid, methyl ester |
Others | Others |
Methyl retinoate (Retinoic acid, methyl ester) 在体内实验中会诱导给部位发生无菌炎症,会使血液中白细胞数量增加,而红细胞和Hb含量减少。Methyl retinoate 可促进自发性白血病的产生。Methyl retinoate 可用于治疗因维生素A缺乏而产生的胃肥大和溃疡。 | |||
T31859 |
Fosdenopterin
|
||
Fosdenopterin is a molybdenum cofactor deficiency. | |||
T32193 |
Iron sorbitex
Jectofer,Yectofer,Iron Sorbitol,HSDB 1967,Astra 1572 |
||
Iron Sorbitex is used for the treatment of Iron Sorbitex deficiency anemia in patients with chronic kidney disease. Iron Sorbitex can be used as an Iron Sorbitex replacement product used for the treatment of Iron Sorbitex deficiency anemia in patients wit | |||
T73783 | D-Ribofuranose | ||
D-Ribofuranose (D-Ribose) 是一种在脑脊液中发现的内源性代谢物,用于磷酸 5 核糖异构酶缺乏症和中链酰基 CoA 脱氢酶缺乏症的研究。 | |||
T1182L |
Levocarnitine propionate
L-Propionylcarnitine,ST261,ST 261,Propionyl-L-carnitine,ST-261 |
||
Propionylcarnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency. | |||
T34316 |
Rhizoferrin
|
Others | Others |
Rhizoferrin 是一种来自真菌Rhizopus microsporus var.rhizopodiformis 的新型铁载体,可用于研究植物缺铁引起的萎黄病。 | |||
T69677 | Mitapivat sulfate anhydrous | ||
Mitapivat sulfate anhydrous is an allosteric activator of Pyruvate Kinase (PK) that may be useful in the treatment of Pyruvate Kinase Deficiency. | |||
T31851 |
Formiminoglutamic acid
NSC-334078,NSC334078,NSC 334078 |
||
Formiminoglutamic acid is used to indicate folic acid deficiency and megaloblastic anemia of pregnancy with the measurement in the urine after oral administration of histidine. | |||
T61512 | FT3967385 | ||
FT3967385 is a newly developed compound that functions as a USP30 inhibitor, emulating the genetic deficiency of USP30. This inhibition serves as a catalyst for the amplification of mitochondrial ubiquitylation through the PINK1-PARKIN pathway. | |||
T69865 |
Mitapivat sulfate
|
||
Mitapivat sulfate, also known as AG-348 sulfate hydrate, is an allosteric activator of Pyruvate Kinase (PK) that may be useful in the treatment of Pyruvate Kinase Deficiency. | |||
T15709 |
Lapaquistat acetate
TAK-475 |
Others | Others |
Lapaquistat acetate is a squalene synthase inhibitor. It also blocks the conversion of farnesyl diphosphate (FPP) to squalene. Lapaquistat acetate is used to Mevalonate Kinase Deficiency (MKD), it is effective at lowering low-density lipoprotein cholester | |||
T73734 |
Acetoacetic acid
|
||
Acetoacetic acid是一种存在于脑脊液中的内源代谢物,主要应用于脑膜炎、妊娠、3-羟基-3-甲基戊二酰辅酶A裂解酶缺乏症、子痫前期/子痫、2型糖尿病、1型葡萄糖转运蛋白缺乏综合征及琥珀酰辅酶A:3-氧酸辅酶A转移酶缺乏症的研究中。 | |||
T82294 | GLUT1-IN-3 | ||
GLUT1-IN-3 (Compd 4b) 是针对1型葡萄糖转运蛋白缺乏综合症相关疾病研究的化合物,具有有效抑制癫痫发作的作用。 | |||
T64308 |
Fosdenopterin hydrobromide dihydrate
|
||
Fosdenopterin (Precursor Z) hydrobromide dehydrate 是一种合成的环吡喃蝶呤单磷酸盐 (cPMP),能够用于A 型钼辅因子缺乏症 (MoCD) 的研究。 | |||
T76121 |
Phosphoglucomutase
|
Endogenous Metabolite | Metabolism |
Phosphoglucomutase可促进弓形虫的囊肿发育。缺乏 Phosphoglucomutase 会引发代谢疾病。 | |||
T61669 | BChE-IN-4 | ||
BChE-IN-4 is a highly potent inhibitor of the enzyme butyrylcholinesterase, capable of crossing the blood-brain barrier. It effectively reduces learning and memory impairments resulting from a deficiency in cholinergic activity in a mouse model. BChE-IN-4 holds promise for further investigation in the field of Alzheimer's disease research [1]. | |||
T36842 |
Isobutyryl Coenzyme A (sodium salt)
|
||
Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA. Isobutyryl-CoA is a substrate for isobutyryl-CoA dehydrogenase (IBDH) in the catabolism of valine and an intermediate in the synthesis of isobutyryl-L-carnitine , which accumulates in IBDH deficiency. | |||
T73790 | 3-Hydroxysebacic acid | ||
3-Hydroxysebacic acid 是存在于尿中的内源代谢物,可用于研究中链酰基钴 A 脱氢酶缺乏症。 | |||
T36947 |
Pyrithiamine (hydrobromide)
|
||
Pyrithiamine is the pyridine analog of thiamine that prevents growth of organisms that require intact thiamine. [1] It inhibits the growth of bacterial and fungal species at a pyrithiamine:thiamine ratio of 10:1 in growth media and induces symptoms of thiamine deficiency in mice at a dietary ratio of 3:1. These effects are reversible with addition of sufficient thiamine in all species. Pyrithiamine inhibits the formation of cocarboxylase from thiamine in chicken blood in a dose-dependent manner.... | |||
T15691 |
L-Alanosine
NSC-153353,丙氨菌素 |
Others | Others |
L-Alanosine inhibits adenylosuccinate synthetase. L-Alanosine is an antibiotic from Streptomyces alanosinicus with antineoplastic activity. L-Alanosine blocks the common de novo purine biosynthesis pathway and, thereby, inhibit tumor cells with MTAP defic | |||
T72973 |
Octotiamine
硫辛酸硫胺,TATD |
||
Octotiamine (TATD),一种维生素B1类似物,成为维生素复合物的组成部分,主要用于研究维生素B1缺乏症。 | |||
T63892 |
RAD51-IN-5
|
||
RAD51-IN-5 是 RAD51 有效的抑制剂。其中 RAD51 是一种真核生物基因。RAD51-IN-5 对线粒体缺陷病症表现出研究潜力。 | |||
T73754 |
Pristanic acid
|
||
Pristanic acid,一种血液中的内源代谢物,用于研究α甲基酰基辅酶A消旋酶缺乏症和齐薇格综合征。 | |||
T63728 |
RAD51-IN-7
|
||
RAD51-IN-7 是 RAD51 的有效抑制剂。其中 RAD51 是一种真核生物基因。RAD51-IN-7 对线粒体缺陷病症表现出研究潜力。 | |||
T35665 |
Pregnanetriol
|
||
Pregnanetriol is a metabolite of 17α-hydroxyprogesterone .1,2It is formed from 17α-hydroxyprogesterone by reduction of the C-20 ketone.2Urinary levels of pregnanetriol are elevated in patients with 21-hydroxylase deficiency and congenital adrenal hyperplasia.3,4 1.Kamrath, C., Hartmann, M.F., Boettcher, C., et al.Diagnosis of 21-hydroxylase deficiency by urinary metabolite ratios using gas chromatography-mass spectrometry analysis: Reference values for neonates and infantsJ. Steroid Biochem. Mol... | |||
T75328 | Hydroxocobalamin hydrochloride | ||
Hydroxocobalamin hydrochloride 是天然维生素B12的可注射形式,因其优良的安全性质,被用于研究维生素B12缺乏症(包括恶性贫血)相关治疗。 | |||
T73774 | (S)-3,4-Dihydroxybutyric acid lithium hydrate | ||
(S)-3,4-Dihydroxybutyric acid (lithium hydrate) 为一种在人体尿液中自然出现的代谢物,其在琥珀酸半醛脱氢酶(SSADH)缺乏症患者体内的浓度会出现上升。 |
Cat. No. | Product Name | Target | Signaling Pathways |
---|---|---|---|
T0613 |
3-Hydroxyisovaleric acid
β-羟基异戊酸,Beta-Hydroxyisovaleric acid |
Others; Endogenous Metabolite | Metabolism; Others |
3-Hydroxyisovaleric acid (Beta-Hydroxyisovaleric acid) 是尿液中排泄的内源性代谢产物 ,可作为生物素缺乏的早期和敏感指标。 | |||
T0062 |
Folic acid
Folate,Vitamin B9,Pteroylglutamic acid,Vitamin M,叶酸,Folacin |
DNA/RNA Synthesis; Endogenous Metabolite | Cell Cycle/Checkpoint; DNA Damage/DNA Repair; Metabolism |
Folic acid (Vitamin B9) 是维生素B9,是合成DNA/RNA,新细胞产生和维持的必要成分。 | |||
T4734 |
Suberic acid
Octanedioic acid,1,8-Octanedioic acid,辛二酸 |
Others; Endogenous Metabolite | Metabolism; Others |
Suberic acid (1,8-Octanedioic acid) 被发现和肉毒碱-甲酰基-丙氨酸转位酶缺乏(丙二醇-羧基酶缺乏症)有关。 | |||
T19581 |
Tiglyl carnitine
|
Endogenous Metabolite | Metabolism |
Tiglyl carnitine 与肺癌和肉碱和甘氨酸对3-酮硫解酶缺乏症有关 。 | |||
T3S0507 |
Homovanillic acid
Homovaniuic acid,Vanilacetic acid,高香草酸,Vanillacetic acid |
Others; Endogenous Metabolite | Metabolism; Others |
Homovanillic acid (Vanillacetic acid) 是一种多巴胺代谢物,它与乳糜泻、生长激素缺乏、芳香族氨基酸脱羧酶缺乏症以及脂联素还原酶缺乏症相关。 | |||
T4851 |
Methyl acetylacetate
Methyl acetoacetate,乙酰乙酸甲酯 |
Others; Endogenous Metabolite | Metabolism; Others |
Methyl acetylacetate (Methyl acetoacetate) 是内源性代谢产物的一种。 | |||
T5279 |
Ethylmalonic acid
alpha-Carboxybutyrate,2-Ethylmalonic acid,乙基丙二酸 |
Others; Endogenous Metabolite | Metabolism; Others |
Ethylmalonic acid (alpha-Carboxybutyrate) 是潜在有毒物质,非致癌性,与神经性厌食和甲氧基脱羧酶缺乏症有关。 | |||
T5273 |
2-Hydroxy-3-methylbutanoic acid
2-Hydroxy-3-methylbutyric acid,2-Hydroxyisovaleric Acid,2-羟基-3-甲基丁酸,(±)-2-Hydroxyisopentanoic Acid |
Others; Endogenous Metabolite | Metabolism; Others |
2-Hydroxy-3-methylbutanoic acid ((±)-2-Hydroxyisopentanoic Acid) 是一种 GHB 的结构类似物,其中GHB 是一种中枢神经系统中发现的天然物质,也是一种神经药物。 | |||
T4766 |
3-Methylglutaric acid
|
Endogenous Metabolite | Metabolism |
3-Methylglutaric acid 是亮氨酸代谢物,是一种与两种亮氨酸途径 HMGCL、AUH 酶缺陷显著相关的 C6 二元羧酸有机酸。 | |||
TQ0024 |
3-Hydroxyglutaric acid
|
Endogenous Metabolite | Metabolism |
3-Hydroxyglutaric acid 是一种戊二酸的衍生物。 | |||
T19608 |
Zinc sulfate heptahydrate
|
Others | Others |
Zinc sulfate heptahydrate 是七水合形式的硫酸锌,是一种膳食补充剂,可用于锌缺乏症和预防高危人群的锌缺乏症。 | |||
T1709 |
2'-Deoxyinosine
Guanine deoxyriboside,Deoxyinosine,2'-脱氧肌苷 |
Others; Endogenous Metabolite | Metabolism; Others |
2'-Deoxyinosine (Guanine deoxyriboside) 具有 抑制人癌细胞株生长的活性,并发现其与嘌呤核苷磷酸化酶缺乏有关。 | |||
T4817 |
Methylmalonic acid
NSC 25201,MMA,Isosuccinic acid,甲基丙二酸,Methylpropanedioic acid,Methylmalonate |
Others; Endogenous Metabolite | Metabolism; Others |
Methylmalonic acid (Isosuccinic acid) 是癌症患者缺乏维生素 B-12的指标。 | |||
TWP2911 |
Thymidine
beta-胸苷,5-Methyldeoxyuridine,Deoxyribothymidine,DThyd,NSC 21548,Thymidin |
Others; DNA/RNA Synthesis; Endogenous Metabolite | Cell Cycle/Checkpoint; DNA Damage/DNA Repair; Metabolism; Others |
Thymidine (DThyd) 是一种细胞同步剂,是脱氧核糖核酸的特殊前体。它可在 DNA 复制前在 G1/S 边界阻滞细胞,是DNA 合成抑制因子。 | |||
T4804 |
Pyruvic acid
BTS,Pyroracemic acid,α-Ketopropionic acid,2-Oxopropionic acid,Acetylformic acid,丙酮酸,NSC 179 |
Others; Endogenous Metabolite | Metabolism; Others |
Pyruvic acid (Acetylformic acid) 是碳水化合物,是蛋白质和脂肪代谢的中间产物。 | |||
T4586 |
(Rac)-Arnebin 1
β,β-二甲基丙烯酰阿卡宁,beta, beta-dimethylacrylshikonin |
Others | Others |
(Rac)-Arnebin 1 (beta, beta-dimethylacrylshikonin) 具有抗肿瘤作用,是β,β-Dimethylacrylalkannin 和 β,β-Dimethylacrylshikonin 的消旋体。β,β-Dimethylacrylalkannin 和 β,β-Dimethylacrylshikonin 是分离自紫草属植物中的萘醌类化合物。 | |||
T17048 |
Tetradecanoylcarnitine
L-Myristoylcarnitine,Myristoyl-L-(-)-carnitine |
Endogenous Metabolite | Metabolism |
Tetradecanoylcarnitine (L-Myristoylcarnitine) 是一种酰基肉碱,参与长链脂肪酸的β-氧化,是极长链酰辅酶A脱氢酶缺乏症的一种潜在标记物。 | |||
T4880 |
L-Dihydroorotic acid
|
Others; Endogenous Metabolite | Metabolism; Others |
L-Dihydroorotic acid 是一种能够通过二氢乳清酶,能够逆水解产生的无环 L-脲基琥珀酸。 | |||
T7094 |
Levomefolic Acid
L-5-甲基四氢叶酸,5-MTHF,5-甲基四氢叶酸 |
Reactive Oxygen Species; Endogenous Metabolite | Immunology/Inflammation; Metabolism; NF-κB |
Levomefolic Acid (5-MTHF) 是一种具有口服活性、可穿过血脑屏障的天然活性叶酸,是广泛使用的叶酸食品补充剂。 | |||
T4894 |
L-Arabinitol
贝他米松-21-丙酸酯,L-(-)-Arabitol |
Endogenous Metabolite | Metabolism |
L-Arabinitol (L-(-)-Arabitol) 是一种红薯、蛾豆、鹿莓等食品的潜在生物标志物,与阿尔茨海默病和 5-磷酸核糖异构酶缺乏症有关。 | |||
TN1957 |
Mulberrofuran Q
MulberrofuranQ |
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Mulberrofuran Q是一种有活性的化合物,可抑制12-羟基-5,8,10-十七碳三烯酸(HHT)和血栓烷B2(环加氧酶产物)的形成 ,可保护神经细胞免受缺氧-缺糖(OGD)诱导的氧化应激的影响。 | |||
T5233 |
N-Methylnicotinamide
N'-Methylnicotinamide,Nicotinic Acid Methylamide,N-Methylpyridine-3-carboxamide,N-甲基烟酰胺 |
Others; Endogenous Metabolite | Metabolism; Others |
N-Methylnicotinamide (Nicotinic Acid Methylamide) 是内源性代谢产物的一种。 | |||
T5515 |
Hemin
氯化血红素,Hemin chloride |
Mitophagy; Ferroptosis; Autophagy | Apoptosis; Autophagy |
Hemin (Hemin chloride) 是一种含有氯的含铁卟啉,一种血红素加氧酶 (HO)-1 诱导剂。Hemin 对卟啉病具有治疗活性,可以减少患者的血红素缺乏,从而通过生物化学反馈抑制 δ-氨基乙酰丙酸合成酶的活性。 | |||
T5291 |
Malonic acid
Carboxyacetic Acid,1,3-Propanedioic Acid,丙二酸,Propanedioic Acid |
Others | Others |
Malonic acid (propanedioic acid) 是竞争性抑制剂的典型例子:它作用于呼吸电子传递链中的琥珀酸脱氢酶(复合物 II)。发现丙二酸与丙二酰辅酶A 脱羧酶缺乏有关,这是一种先天性代谢错误。 | |||
T4887 |
3-Amino-2-methylpropanoic acid
DL-3-AMINOISOBUTYRIC ACID,3-氨基异丁酸,α-Methyl-β-alanine |
Endogenous Metabolite | Metabolism |
3-Amino-2-methylpropanoic acid (α-Methyl-β-alanine) 是 N-carbamyl-beta-aminoisobutyric acid 经 enzyme Beta-ureidopropionase (EC 3.5.1.6)转化后的产物,是嘧啶降解的最后一步。-尿嘧啶丙酸酶缺乏症是一种与神经异常相关的嘧啶降解的先天性错误。 | |||
T5076 |
L-Allothreonine
L-别苏氨酸,L-allo-Threonine |
Others; Endogenous Metabolite | Metabolism; Others |
L-Allothreonine (L-allo-Threonine) 是内源性代谢产物的一种。 | |||
T3O2749 |
L-Leucine
(S)-Leucine,Leu,L-亮氨酸,亮氨酸 |
mTOR | PI3K/Akt/mTOR signaling |
L-Leucine ((S)-Leucine) 是必需的支链氨基酸,能够激活 mTOR 信号通路。 | |||
T4856 |
Acetyl-L-lysine
N-乙酰-L-赖氨酸,Nα-Acetyl-L-lysine,(2S)-2-Acetamido-6-aminohexanoic acid |
Endogenous Metabolite | Metabolism |
Acetyl-L-lysine (Nα-Acetyl-L-lysine) 是一种 N-乙酰化氨基酸。它是人类尿液中的一种正常成分,在正常样本中的浓度太小,无法进行常规检测;然而,在一名氨基酰化酶I缺乏症患者的尿液中发现其水平升高。 | |||
T4901 |
5'-Guanylic acid disodium salt
Guanosine 5'-monophosphate disodium salt,5'-鸟苷酸二钠,鸟苷钠5'-单磷酸,GMP-5 |
Others; Endogenous Metabolite | Metabolism; Others |
5'-Guanylic acid disodium salt (GMP-5) 由鸟嘌呤,核糖和磷酸组成,是信使 RNA 中的一种核苷酸单体。 鸟苷衍生物参与细胞内信号转导,并已鉴定存在于端粒,核糖体 DNA,免疫球蛋白重链转换区和原癌基因的控制区中的重复基因组序列中。 | |||
T3578 |
Pyridoxal phosphate
PLP,PAL-P,磷酸吡哆醛,pyridoxal 5'-phosphate,Pyridoxyl phosphate,Pyridoxal 5′-phosphate,Vitamin B6 phosphate |
Reverse Transcriptase; Endogenous Metabolite | Metabolism; Microbiology/Virology |
Pyridoxal phosphate (Vitamin B6 phosphate) 是维生素 B6 的活性形式,可抑制逆转录酶活性,用于研究迟发性运动障碍症。 | |||
T4858 |
4-Hydroxyphenylpyruvic acid
4-羟苯基丙酮酸,4-Hydroxyphenylpyruvic acid |
Endogenous Metabolite | Metabolism |
4-Hydroxyphenylpyruvic acid 是一种苯丙氨酸代谢的中间产物,是一种酶抑制剂。 | |||
T5267 |
N-Acetyl-L-glutamic acid
Ac-L-Glu-OH,N-乙酰-L-谷氨酰胺,N-Acetylglutamic acid |
Others; Endogenous Metabolite | Metabolism; Others |
N-Acetyl-L-glutamic acid (Ac-L-Glu-OH) 是一种 N-Acyl-L-氨基酸,是动物细胞培养基的组成成分,是一种酿酒酵母和人类的代谢物。 | |||
T5215 |
N-(3-Phenylpropionyl)glycine
N-(3-苯基丙酰基)甘氨酸,Phenylpropionylglycine |
Others | Others |
N-(3-Phenylpropionyl)glycine (Phenylpropionylglycine) 是一种酰基甘氨酸。酰基甘氨酸通常是脂肪酸的次要代谢物。 | |||
T1040 |
Vitamin B12
Cyanocobalamin,Cobalamin,维生素B12 |
Endogenous Metabolite | Metabolism |
Vitamin B12 (Cobalamin) 是一种维生素,在维持血流形成和脑、神经系统正常功能等方面发挥关键作用。 | |||
T4947 |
3-Hydroxybutyric acid
Butanoic acid,3-羟基丁酸 |
Endogenous Metabolite | Metabolism |
3-Hydroxybutyric acid (Butanoic acid) 是一种人内源性代谢物,在 I 型糖尿病中升高。它能够调节膜脂的性质。 | |||
T4919 |
Ureidopropionic acid
Carbamoyl-b-alanine,3-酰脲丙酸,3-Ureidopropionic acid |
Endogenous Metabolite | Metabolism |
Ureidopropionic acid (3-Ureidopropionic acid) 是尿嘧啶代谢的中间体。更具体地说,它是二氢尿嘧啶的分解产物,由二氢嘧啶酶产生。它通过β -脲丙酸酶进一步分解为β -丙氨酸。尿素丙酸本质上是β -丙氨酸的尿素衍生物。高水平的尿丙酸存在于β -尿丙酸酶(UP)缺乏的个体中。嘧啶代谢中的酶缺乏与抗肿瘤药物5-氟尿嘧啶的严重毒性风险有关。 | |||
T4827 |
Trimethylammonium chloride
三甲胺盐酸盐,Trimethylamine hydrochloride |
Others; Endogenous Metabolite | Metabolism; Others |
Trimethylammonium chloride (Trimethylamine hydrochloride) 是动植物分解的产物。它是造成鱼腥味的主要物质,通常与脏鱼、细菌性阴道感染和口臭有关。它也与服用大剂量胆碱有关。三甲胺尿是一种遗传性疾病,身体无法从食物中代谢三甲胺。食用富含胆碱的食物后,患者的汗液、尿液和呼吸中会出现特有的鱼腥味。三甲胺尿症是一种常染色体隐性遗传病,涉及三甲胺氧化酶缺乏症。三甲氨基尿症在罗德岛红鸡中也有发现,这种鸡产的蛋有腥味。尿液中的 Trimethylammonium chloride 是食用豆类的生物标志物。 | |||
T4838 |
(S)-Malic acid
L-(-)-Malic acid,(S)-(-)-HYDROXYSUCCINIC ACID,(S)-2-Hydroxysuccinic acid,L-苹果酸 |
Others; Endogenous Metabolite | Metabolism; Others |
(S)-Malic acid ((S)-2-Hydroxysuccinic acid) 是天然存在的二羧酸,是水果酸甜味的来源,常用作食品添加剂。 | |||
T4776 |
Glycerol
Glycerin,甘油 |
Endogenous Metabolite | Metabolism |
Glycerol 是甘油三酯(即脂肪和油)和磷脂的重要成分。它在食品工业中被广泛用作甜味剂和保湿剂以及药物制剂。 | |||
T39460 |
Mead acid
5,8,11-Eicosatrienoic acid |
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Mead acid, also known as 5,8,11-Eicosatrienoic acid, is an unsaturated fatty acid belonging to the Omega-9 series. It serves as an indicator of deficiency in essential fatty acids. | |||
T19346 |
5'-Guanylic acid
鸟苷酸,5'-guanosine monophosphate,5'-GMP |
Endogenous Metabolite | Metabolism |
5'-Guanylic acid (5'-guanosine monophosphate) 与几种代谢紊乱有关,包括腺苷脱氨酶缺乏症、 aica-核糖体途径、腺嘌呤磷酸转移酶缺乏症 (aprt) 以及 2-羟基葡糖酸盐途径。 | |||
T13731 |
Hydroxocobalamin monohydrochloride
Vitamin B12a monohydrochloride,盐酸羟钴胺 |
Others | Others |
Hydroxocobalamin monohydrochloride is an injectable naturally occurring form of vitamin B12. It is used as a dietary supplement in the treatment of vitamin B12 deficiency including pernicious anemia. | |||
T37977 |
Succinyladenosine
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Succinyladenosine is a biochemical marker of adenylosuccinase (ASL) deficiency. It is formed through the dephosphorylation of intracellular adenylosuccinic acid (S-AMP) by cytosolic 5-nucleotidase [1]. | |||
T40381 |
2,8-Dihydroxyadenine
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2,8-Dihydroxyadenine, an endogenous metabolite, can induce the generation of urinary crystals and nephrolithiasis. Moreover, 2,8-Dihydroxyadenine serves as a diagnostic tool for detecting adenine phosphoribosyltransferase (APRT) deficiency. | |||
T73775 |
Dopamine 3-O-sulfate
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Dopamine 3-O-sulfate,一种主要存在于血浆中的化合物,可作为芳香族氨基酸脱羧酶(AADC)缺乏症的生物标志物。 | |||
T36472 |
Tetrahydro-11-deoxy Cortisol
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Tetrahydro-11-deoxy cortisol (THS) is the primary urinary metabolite of 11-deoxycortisol. Urinary excretion of THS is elevated in patients with 11β-hydroxylase deficiency, a condition resulting from mutations in the cytochrome P450 (CYP) isoform CYP11B1. Urinary levels of THS are also elevated in patients with adrenocortical carcinoma (ACC) and adrenocortical adenoma (ACA) but are higher in patients with ACC compared to ACA. | |||
T37177 |
Norcholic Acid
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Norcholic acid is a bile acid and 23-carbon derivative of cholic acid .1Levels of norcholic acid are increased in the urine of patients with liver cirrhosis or cerebrotendinous xanthomatosis (CTX), an inborn error of metabolism characterized by a deficiency in the mitochondrial enzyme sterol 27-hydrolylase (CYP27A1) that leads to progressive neurological symptoms.2,3,4 | |||
TN3472 | Axillaridine A | Others | Others |
Axillaridine A is a new cholinesterase inhibitors, it may act as potential leads in the discovery of clinically useful inhibitors for nervous-system disorders, particularly by reducing memory deficiency in Alzheimer's disease patients by potentiating and | |||
T78476 |
Hydroxocobalamin
Vitamin B12a |
Endogenous Metabolite | Metabolism |
Hydroxocobalamin(Vitamin B12a)为可注射型天然维生素B12,不良反应特性良好,适用于治疗维生素B12缺乏症(包括恶性贫血)的膳食补充。 | |||
T78466 |
Erythronic acid potassium
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Endogenous Metabolite | Metabolism |
Erythronic acid potassium为糖类内源性代谢物,关键作用于高尿酸血症发病及改善过程,并与转醛缩酶缺乏症相关的线粒体功能障碍有关,常用于代谢性疾病研究。 | |||
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