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(E)-3-Methylglutaconic acid

品牌
Supelco
CAS
372-42-9
货号
44108
包装型号
规格纯度
≥97.0% (HPLC)
参考价格
2264.11 *本价格含增值税费
促销
数量
-+
产品介绍:

产品说明

生化/生理作用

3-Methylglutaconic aciduria (MGCA) is a group of metabolic disorders characterized by increased urinary excretion of 3-methylglutaconic acid and 3-methylglutaric acid. Five distinct forms of MGCA have been recognized. MGCA type 1 is caused by primary deficiency of the mitochondrial enzyme 3-methylglutaconyl-CoA hydratase (3-MGCH), resulting in a block of leucine degradation. In all other types, the activities of 3-MGCH and other enzymes of leucine degradation are normal and the MGCA is thought to be secondary to a defect in another pathway. MGCA type 2, also known as Barth syndrome, is an X-linked cardiomyopathy associated with skeletal myopathy, neutropenia, and growth retardation. MGCA type 3, also referred to as Costeff optic atrophy syndrome, is an autosomal recessive disorder caused by mutations in the gene OPA3. It is characterized by early-onset bilateral optic atrophy, later-onset extrapyramidal dysfunction. MGCA type 5 caused by mutation in the DNAJC19 gene, is characterized by early-onset dilated cardiomyopathy with conduction defects, nonprogressive cerebellar ataxia, testicular dysgenesis, and growth failure. MGCA type 4 is the unclassified type, which includes all other patients with MGCA.

基本信息

经验(实验)分子式C6H8O4
分子量144.13
Beilstein1722909
MDL编号MFCD01556044
PubChem化学物质编号329756452
NACRESNA.24

产品性质

质量水平100
测定≥97.0% (HPLC)
mp137-143 ℃
application(s)clinical testing
格式neat
储存温度2-8℃
InChI1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+
InChI keyWKRBKYFIJPGYQC-DUXPYHPUSA-N

安全信息

象形图GHS07
警示用语:Warning
危险声明H315 - H319
预防措施声明P264 - P280 - P302 + P352 - P305 + P351 + P338 - P332 + P313 - P337 + P313
危险分类Eye Irrit. 2 - Skin Irrit. 2
储存分类代码11 - Combustible Solids
WGKWGK 3
闪点(F)Not applicable
闪点(C)Not applicable

SUPELCO

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