推荐产品
应用
4,6-二氧庚酸已用于:
- 作为树突状细胞条件培养基组分并抑制树突状细胞中的ALA脱水酶(ALAD)(50)
- 作为人单个核细胞(51)和HeLa细胞(52)中的血红素的生物合成抑制剂
- 作为恶性疟原虫培养物中的琥珀酰丙酮的定量标准品(53)
生化/生理作用
4,6-二氧庚酸是一种血红素生物合成抑制剂。它是酪氨酸分解的代谢副产物,可抑制δ氨基酮戊酸(ALA)脱水酶。它是检测酪氨酸血症Ⅰ型的潜在标志物,是新生儿干血斑分析的检测目标。
血红素生物合成抑制剂
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
dust mask type N95 (US), Eyeshields, Gloves
JIMD reports, 53(1), 90-102 (2020-05-13)
Nitisinone is used to treat hereditary tyrosinemia type 1 (HT-1) by preventing accumulation of toxic metabolites, including succinylacetone (SA). Accurate quantification of SA during newborn screening is essential, as is quantification of both SA and nitisinone for disease monitoring and
Succinylacetone as primary marker to detect tyrosinemia type I in newborns and its measurement by newborn screening programs
Molecular Genetics and Metabolism, 113(1-2), 67-75 (2014)
Hong Kong medical journal = Xianggang yi xue za zhi, 24(4), 340-349 (2018-03-03)
Hereditary channelopathies and cardiomyopathies are potentially lethal and are clinically and genetically heterogeneous, involving at least 90 genes. Genetic testing can provide an accurate diagnosis, guide treatment, and enable cascade screening. The genetic basis among the Hong Kong Chinese population
lonp1-dependent Breakdown Of Mitochondrial 5-aminolevulinic Acid Synthase Protein By Heme In Human Liver Cells: 904
Hepatology, 54(4), 785A-785A (2011)
Molecular genetics and metabolism, 88(1), 16-21 (2006-02-02)
Tyrosinemia type I (TYR 1) is a severe disorder causing early death if left untreated. While tyrosine can be determined in dried blood spots (DBS), it is not a specific marker for TYR 1 and most often associated with benign
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