推荐产品
质量水平
检测方案
≥97.0% (TLC)
形式
powder, crystals or chunks
旋光性
[α]/D -23±2°, c = 1 in H2O
颜色
white to off-white
储存温度
2-8°C
SMILES字符串
C[N+](C)(C)C[C@H](OC(CCC)=O)CC([O-])=O
InChI
1S/C11H21NO4/c1-5-6-11(15)16-9(7-10(13)14)8-12(2,3)4/h9H,5-8H2,1-4H3/t9-/m1/s1
InChI key
QWYFHHGCZUCMBN-SECBINFHSA-N
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应用
- Lead exposure induces metabolic reprogramming in rat models.: This study by Mani MS et al. investigates the metabolic effects of lead exposure in rat models, focusing on the biochemical pathways affected. The research highlights the role of butyryl-ʟ-carnitine in counteracting lead-induced metabolic disruptions, suggesting its potential therapeutic applications in mitigating heavy metal toxicity (Mani et al., 2020).
生化/生理作用
酰基辅酶A脱氢酶,短链(SCAD)缺乏症患者,急性酸中毒和全身性肌肉无力的婴儿以及肌肉局部慢性肌病的中年患者的丁酰肉碱升高;极长链酰基辅酶A脱氢酶缺乏症和乳糜泻。
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
International journal of andrology, 6(4), 349-357 (1983-08-01)
Bioautography of human semen demonstrated the presence of L-carnitine, acetylcarnitine, propionylcarnitine and C4-acylcarnitines (butyrylcarnitines). In studies designed to ascertain the organs secreting these compounds into semen it was found that: Quantitative analyses of semen obtained pre- and post-vasectomy showed markedly
Clinica chimica acta; international journal of clinical chemistry, 337(1-2), 103-113 (2003-10-22)
Homozygosity and compound heterozygosity for the short chain acyl-CoA dehydrogenase (SCAD) gene sequence variants 625G-->A and 511C-->T are associated with ethylmalonic aciduria (EMA), a biochemical indicator of SCAD deficiency. The clinical and biochemical implications of these variants are not fully
The Journal of pediatrics, 126(6), 910-915 (1995-06-01)
We identified two additional patients with short-chain acyl-coenzyme A (CoA), further characterized the clinical and biochemical features of this defect, and compared it with other fatty acid oxidation defects. We have measured the in vitro short-chain acyl-coenzyme A dehydrogenase (SCAD)
Metabolites, 8(4) (2018-10-05)
Duchenne muscular dystrophy (DMD) is a musculoskeletal disorder that causes severe morbidity and reduced lifespan. Individuals with DMD have an X-linked mutation that impairs their ability to produce functional dystrophin protein in muscle. No cure exists for this disease and
Journal of lipid research, 38(1), 173-182 (1997-01-01)
A stable isotope dilution gas chromatography chemical ionization mass spectrometry (GC-CI-MS) method was developed for the quantitative profiling of plasma acylcarnitines. The clean-up procedure was comprised of a solid-phase cation exchange extraction using PRS-columns from which the acylcarnitines were eluted
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