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用途
sufficient for 200 reactions (Fluorometric)
檢測方法
fluorometric
相關疾病
cancer; neurological disorders
運輸包裝
wet ice
儲存溫度
−20°C
一般說明
D-2-羟基戊二酸(D2HG)在正常细胞和组织中含量偏低，但在罕见常染色体病D2HG酸尿症等代谢病症中浓度明显升高。在其他代谢疾病中，D2HG浓度略有上升，包括多种酰基辅酶A脱氢酶缺乏症、二氢硫辛酸脱氢酶缺乏症、丙酮酸脱羧酶缺乏症和丙酮酸羧化酶缺乏症、多种癌症、以及异柠檬酸脱氢酶1(IDH1)和异柠檬酸脱氢酶2(IDH2)基因突变的肿瘤。D2HG浓度升高检测是针对这些疾病开展早期诊断、预后、监测和制定治疗策略的重要生物标志物。
特點和優勢
- 高灵敏度、快速的酶法测定各种生物液体中的D2HG浓度
- 辅助计算器（点击此处下载计算器excel文件） 根据您的实验数据分析结果！
- 快速指示台卡 - 确保您的实验成功
- 一个试剂盒可完成更多实验 - 包含的试剂足够进行 200 次检测
- 检测时间：仅需30-60分钟
適合性
适用于检测细胞/组织裂解液、血清、尿液、培养细胞和培养上清液中的D2HG浓度。
原則
与德国癌症研究中心(DKFZ)合作开发，D-2-羟基戊二酸分析试剂盒是快速、灵敏地测定血清、尿液、细胞培养上清和细胞/组织裂解液等各种生物液体中D2HG浓度的酶分析法。这种检测法最初由Balss等开发，8原理是(D)-2-羟基戊二酸脱氢酶(HGDH)催化D2HG氧化成为α-酮戊二酸(αKG)，同时伴随NAD+还原为NADH（见图1）。形成的NADH可通过黄递酶介导的将刃天青还原为荧光染料试卤灵的反应进行定量(λ激发 = 540 nm/λ发射 = 590 nm)。
法律資訊
本产品根据德国癌症研究中心(DKFZ)和University Clinic of Heidelberg（海德堡大学医院）的许可售卖。本产品的使用受到美国专利及外国专利声明保护，包括美国专利No. 9,487,815，欧洲专利No. 2 820 145和其他外国对应专利。本品仅供研究使用。
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
其他客户在看
R A Chalmers et al.
Journal of inherited metabolic disease, 3(1), 11-15 (1980-01-01)
A patient with protein-losing gastroenteropathy and egg allergy has been shown to have a previously unrecognized organic aciduria, D-2-hydroxyglutaric aciduria. The observations made are consistent with an inherited metabolic disorder in the catabolism of 5-aminolaevulinate possibly due to deficient activity
Roberta Leonardi et al.
The Journal of biological chemistry, 287(18), 14615-14620 (2012-03-24)
Isocitrate dehydrogenase (IDH) is a reversible enzyme that catalyzes the NADP(+)-dependent oxidative decarboxylation of isocitrate (ICT) to α-ketoglutarate (αKG) and the NADPH/CO(2)-dependent reductive carboxylation of αKG to ICT. Reductive carboxylation by IDH1 was potently inhibited by NADP(+) and, to a
Eduard A Struys et al.
American journal of human genetics, 76(2), 358-360 (2004-12-21)
d-2-hydroxyglutaric aciduria is a neurometabolic disorder with both a mild and a severe phenotype and with unknown etiology. Recently, a novel enzyme, d-2-hydroxyglutarate dehydrogenase, which converts d-2-hydroxyglutarate into 2-ketoglutarate, and its gene were identified. In the genes of two unrelated
R A Chalmers et al.
Clinica chimica acta; international journal of clinical chemistry, 77(2), 117-124 (1977-06-01)
Detailed studies, using gas chromatography and mass spectrometric methods, of the urinary organic acids excreted by a patient with proven pyruvate decarboxylase deficiency are reported. In addition to the greatly-increased levels of lactate and pyruvate, marked elevation in the levels
S I Goodman et al.
Pediatric research, 14(1), 12-17 (1980-01-01)
When amino acids were infused at a rate of 4 g/kg/day, an infant with hypoglycemia, metabolic acidemia and chronic regurgitation showed hypersarcosinemia and excreted abnormal amounts of sarcosine, isovalerylglycine, isobutyrylglycine, alpha-methylbutyrylglycine, and beta-hydroxyisovaleric, glutaric, alpha-hydroxyglutaric, methylsuccinic, and alpha-hydroxyisobutyric acids in
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