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Search Results for " aciduria "

Targets Recommended: Others

2

抑制剂 & 化合物

12

天然产物

1

重组蛋白

4

同位素标记化合物

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Cat. No.	Product Name	Target	Signaling Pathways
T73752	Glutarylcarnitine lithium	Others	Others
	Glutarylcarnitine lithium 丙二酸尿症和 I 型戊二酸尿症的重要诊断代谢物。
T78071	3-Methylglutaconic acid β-Methylglutaconic acid	GABA Receptor	Membrane transporter/Ion channel; Neuroscience
	3-Methylglutaconic acid 是MGTA (3-甲基戊二酸尿症) 的主要积累代谢物，能够诱发脂质和蛋白质的氧化损伤，减弱大脑皮层上清液的非酶促抗氧化防御，导致氧化应激。此化合物适用于研究大脑损伤相关疾病。

化合物

Glutarylcarnitine lithium

Cat.No: T73752

Target: Others

3-Methylglutaconic acid

Cat.No: T78071

Synonym: β-Methylglutaconic acid

Target: GABA Receptor

Cat. No.	Product Name	Target	Signaling Pathways
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。
T19340	Glutarylcarnitine O-glutaroyl-L-carnitine	Endogenous Metabolite	Metabolism
	Glutarylcarnitine (O-glutaroyl-L-carnitine) 是大多数串联质谱新生儿筛查计划中监测的丙二酸尿症和谷草酸尿症 I 型的诊断代谢物。
T5550	2-Hydroxy-2-methylbutanoic acid	Others; Endogenous Metabolite	Metabolism; Others
	2-Hydroxy-2-methylbutanoic acid 是非正常的代谢物，与羟基戊二酸尿症以及枫糖尿病有关。
T5305	3-Methylbut-2-enoic acid 异戊烯酸,3,3-Dimethylacrylic Acid,Senecioic acid,3-Methylcrotonic Acid	Others; Endogenous Metabolite	Metabolism; Others
	3-Methylbut-2-enoic acid (Senecioic acid) 是内源性代谢产物的一种。
T5246	Trans-2-butene-1,4-dicarboxylic acid trans-2-Butene-1,4-dicarboxylic Acid,3-Hexenedioic Acid,trans-3-Hexenedioic Acid,β-Hydromuconic Acid,反式-2-丁烯-1,4-二甲酸	Others; Endogenous Metabolite	Metabolism; Others
	Trans-2-butene-1,4-dicarboxylic acid (3-Hexenedioic Acid) 是内源性代谢产物的一种。
T4861	trans-Aconitic acid	ATPase; Endogenous Metabolite	Membrane transporter/Ion channel; Metabolism
	trans-Aconitic acid 是反式丙酮酸2-甲基转移酶的底物，存在于正常人尿液中，大量存在于 Reye 综合征和有机酸尿症。
T5232	3-Methyl-2-oxobutanoic acid 3-甲基-2-氧丁酸,2-Oxoisovaleric acid,alpha-Ketoisovaleric acid	Others; Endogenous Metabolite	Metabolism; Others
	3-Methyl-2-oxobutanoic acid (2-Oxoisovaleric acid) 是大肠杆菌中泛酸的前体。它是由支链氨基酸的不完全分解产生的一种异常代谢物。
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。
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。
T19289	DL-Glyceric Acid Glyceric Acid (20% in Water,ca.2 mol/L)	Others	Others
	DL-glyceric acid is a compound that is excessively secreted in the urine of patients with D-glyceric aciduria.
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。
T38954	L-Glyceric acid sodium	Others	Others
	L-Glyceric acid sodium is a urinary metabolite predominantly found in individuals with the rare inherited metabolic disorder, L-glyceric aciduria. This compound serves as a diagnostic tool for identifying primary hyperoxaluria type 2 (PH2) and can be used to differentiate between PH1 and PH2 based on its excretion pattern.

天然产物

3-Methylglutaric acid

Cat.No: T4766

Target: Endogenous Metabolite

Glutarylcarnitine

Cat.No: T19340

Synonym: O-glutaroyl-L-carnitine

Target: Endogenous Metabolite

2-Hydroxy-2-methylbutanoic acid

Cat.No: T5550

Target: Others, Endogenous Metabolite

3-Methylbut-2-enoic acid

Cat.No: T5305

Synonym: 异戊烯酸,3,3-Dimethylacrylic Acid,Senecioic acid,3-Methylcrotonic Acid

Target: Others, Endogenous Metabolite

Trans-2-butene-1,4-dicarboxylic acid

Cat.No: T5246

Synonym: trans-2-Butene-1,4-dicarboxylic Acid,3-Hexenedioic Acid,trans-3-Hexenedioic Acid,β-Hydromuconic Acid,反式-2-丁烯-1,4-二甲酸

Target: Others, Endogenous Metabolite

trans-Aconitic acid

Cat.No: T4861

Target: ATPase, Endogenous Metabolite

3-Methyl-2-oxobutanoic acid

Cat.No: T5232

Synonym: 3-甲基-2-氧丁酸,2-Oxoisovaleric acid,alpha-Ketoisovaleric acid

Target: Others, Endogenous Metabolite

L-Dihydroorotic acid

Cat.No: T4880

Target: Others, Endogenous Metabolite

5'-Guanylic acid disodium salt

Cat.No: T4901

Synonym: Guanosine 5'-monophosphate disodium salt,5'-鸟苷酸二钠,鸟苷钠5'-单磷酸,GMP-5

Target: Others, Endogenous Metabolite

DL-Glyceric Acid

Cat.No: T19289

Synonym: Glyceric Acid (20% in Water,ca.2 mol/L)

Target: Others

5'-Guanylic acid

Cat.No: T19346

Synonym: 鸟苷酸,5'-guanosine monophosphate,5'-GMP

Target: Endogenous Metabolite

L-Glyceric acid sodium

Cat.No: T38954

Target: Others

Species：

Expression System：

Tag：

Cat. No.	Product Name	Species	Expression System
TMPY-04476	MVK Protein, Human, Recombinant (His & GST) POROK3,LRBP,MVLK,MK,mevalonate kinase	Human	Baculovirus Insect Cells
	Mevalonate kinase belongs to the GHMP kinase family, Mevalonate kinase subfamily. It can be found in a wide variety of organisms from bacteria to mammals. Mevalonate kinase may be a regulatory site in the cholesterol biosynthetic pathway. Defects in mevalonate kinase can cause mevalonic aciduria (MEVA). It is an accumulation of mevalonic acid which causes a variety of symptoms such as psychomotor retardation, dysmorphic features, cataracts, hepatosplenomegaly, lymphadenopathy, anemia, hypotonia,...

重组蛋白

MVK Protein, Human, Recombinant (His & GST)

Cat.No: TMPY-04476

Species: Human

Expression System: Baculovirus Insect Cells

Cat. No.	Product Name	Target	Signaling Pathways
TMIJ-0054	Propionic-2,2-d2 acid
	Propionic-2,2-d2 acid 是 Propionic acid 的氘代化合物。Propionic acid 的 CAS 号为 79-09-4。Propionic acid 被广泛用作食品中的抗真菌剂。它以低水平天然存在于乳制品中，并与其他短链脂肪酸 (SCFA) 一起普遍存在于人类和其他哺乳动物的胃肠道中，作为微生物消化碳水化合物的最终产物。在动物体内有显着的生理活性。
TMIJ-0052	Propionicacid-d5
	Propionicacid-d5 是 Propionicacid 的氘代化合物。Propionicacid 的 CAS 号为 79-09-4。Propionic acid 被广泛用作食品中的抗真菌剂。它以低水平天然存在于乳制品中，并与其他短链脂肪酸 (SCFA) 一起普遍存在于人类和其他哺乳动物的胃肠道中，作为微生物消化碳水化合物的最终产物。在动物体内有显着的生理活性。
T35698	Octanoic Acid-13C
	Octanoic acid-13C is intended for use as an internal standard for the quantification of octanoic acid by GC- or LC-MS. Octanoic acid is a medium-chain saturated fatty acid. It has been found in Teleme cheeses made from goat, ovine, or bovine milk.1 Octanoic acid is active against the bacteria S. mutans, S. gordonii, F. nucleatum, and P. gingivalis (IC80s = <125, <125, 1,403, and 2,294 μM, respectively).2 Levels of octanoic acid are increased in the plasma of patients with medium-chain acyl-CoA d...
T36055	Nitisinone-13C6 Nitisinone-13C6
	Nitisinone-13C6is intended for use as an internal standard for the quantification of nitisinone by GC- or LC-MS. Nitisinone is an inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), which converts 4-hydroxyphenylpyruvate (HPPA) to homogentisate in the tyrosine catabolic pathway.1Nitisinone increases urinary levels of HPPA and 4-hydroxyphenyllactate (HPLA) in rats when administered at a dose of 10 mg/kg. Nitisinone (3 mg/kg) prevents the neonatal lethality of fumarylacetoacetate hydrolase (F...

同位素标记化合物

Propionic-2,2-d2 acid

Cat.No: TMIJ-0054

Propionicacid-d5

Cat.No: TMIJ-0052

Octanoic Acid-13C

Cat.No: T35698

Nitisinone-13C6

Cat.No: T36055

Synonym: Nitisinone-13C6

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