Hydroxymethylglutaryl-CoA lyasedoi:10.1007/978-3-642-86605-0_94Dietmar SchomburgGBF — Gesellschaft für Biotechnologische Forschung mbHMargit SalzmannGBF — Gesellschaft für Biotechnologische Forschung mbHSpringer Berlin Heidelberg
Hydroxymethylglutaryl-CoA lyase, mitochondrial (Hmgcl), Recombinant Protein 由上海拜力生物科技有限公司供应,该产品简介:Hydroxymethylglutaryl-CoA lyase, mitochondrial (Hmgcl), Recombinant Protein
3-羟基-3-甲基戊二酰辅酶A裂解酶缺乏症基因检测是对疾病名称为3-hydroxy-3-methylglutaryl-CoA lyase deficiency的遗传代谢性疾病所做的基于基因序列检测分析的、医学临床辅助诊断性检测。该病的英文名称还包括:3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency、3-OH 3-CH3 glutaric aciduria、3-OH 3-methyl...
hydroxymethylglutaryl-CoA lyase activity Pfam Domain Function HMGL-like (PF00682) Signal Regions Not Available Transmembrane Regions Not Available Cellular Location Mitochondrion matrix Gene sequence >lcl|BSEQ0019402|Hydroxymethylglutaryl-CoA lyase, mitochondrial (HMGCL) ATGGCAGCAATGAGGAAGGCGCTTCCGCGGCGACTGGT...
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2) hydroxymethyloglutaryl-CoA lyase 甲羟戊二酸单酰辅酶A裂解酶3) 3-hydroxy-3-methylglutaryl coenzyme A(HMG-CoA)reductase 3-羟基3-甲基戊二酸单酰辅酶A(HMG-CoA)还原酶4) 3-hydroxyl-3-methylglutaryl-coenzyme A (HMG-CoA reductase) 3-羟3-甲基戊二酸单酰-辅酶A (HMG-CoA还原酶)5) hydroxy...
Subsequently, cytosolic ATP-citrate lyase (ACL) cleaves citric acid into acetyl-CoA and oxaloacetate, providing the precursor for the synthesis of FAs and terpenes (Lazar et al., 2018; Ma et al., 2019). Sign in to download hi-res image Fig. 1. Metabolic pathways for the production of ...
L. Genetic complementation analysis of 3-hydroxy-3-methylglutaryl Coenzyme A lyase deficiency in cultured fibroblasts. Am. J. Hum. Genet. 36 (1984) 791–801Sovik O, Sweetman L, Gibson KM, Nyhan WL (1984) Genetic complementation analysis of 3-hydroxy-3-methylglutaryl-coenzymeA lyase deficiency...
The management of pregnancy and delivery in 3-hydroxy-3- methylglutaryl-CoA lyase deficiency. Am J Med Genet A. 2016;170(6):1600-2.Pipitone A, Raval DB, Duis J, et al. The management of preg- nancy and delivery in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. Am J Med Genet A....
Leucine is degraded in six enzymatic steps into acetoacetic acid and in the process acetyl-CoA is generated. The last enzyme in this path is 3-hydroxy-3-methylglutaryl CoA lyase. Deficiency of this enzyme causes acidosis (accumulation of acids) and reduction of sugar in the blood (hypoglycemia...