3-Hydroxyisovalerylcarnitine 是一种肉碱衍生物。血浆中的 3-Hydroxyisovaleryl carnitine 可作为人类生物素缺乏的新型生物标志物,其浓度随着生物素缺乏而增加。生物素缺乏使生物素依赖性 3-methylcrotonyl-CoA 羧化酶活性降低,3-methylcrotonyl-CoA 转换为 3-methylglutaconyl-CoA 的过程受阻、亮氨酸分解代谢途径受损...
Mock DM, Horvath T, Stratton S, Bogusiewicz A, Moran J. Urinary excretion of 3-hydroxyisovaleryl carnitine (3HIAC) in response to a leucine load increases in marginal biotin deficiency. J Nutr. 2011; 141:1925-30.Mock DM, Horvath T, Stratton S, Bogusiewicz A, Moran J. Urinary ...
L-CARNITINE: CLO4 3-HYDROXYISOVALERYL CAS号: 2245711-23-1 英文名: 1-Propanaminium, 3-carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N-dimethyl-N-(methyl-d3)-, (2R)-, perchlorate (1:1) 英文别名: 3-Hydroxyisovaleryl-L-carnitine-d3 ClO4;3-Hydroxyisovaleryl-L-carnitine-d3 ...
(3-hydroxyisovalerylcarnitine)Request For Quotation 3-hydroxyisovalerylcarnitine Basic information More.. Product Name: 3-hydroxyisovalerylcarnitine Synonyms: 3-hydroxyisovalerylcarnitine CAS: MF: C12H23NO5 MW: 261.31472 EINECS: Mol File: Mol File...
Urinary excretion of 3-hydroxyisovaleryl carnitine (3HIA-carnitine) is a functional biomarker of biotin status as it reflects impairment in leucine catabolism caused by reduced activity of the biotin-dependent enzyme 3-methylcrotonyl-CoA carboxylase. As part of a 12-wk controlled feeding study, ...
Background Elevated plasma levels of 3-hydroxyisovaleryl-carnitine (C5OH) and impaired leucine catabolism are frequently observed in newborn screening reports, necessitating consideration of various diseases in the differential diagnosis. This study aimed to analyze different forms of C5OH and explore ...
3-HydroxyisovalerylcarnitineTandem mass spectrometryAcylcarnitinesDried-blood spotsNewborn screeningQuality assuranceThe use of tandem mass spectrometry (MS/MS) for the analysis of amino acids and acylcarnitines from dried-blood spots (DBS) has become routine practice in newborn screening laboratories. ...
Objective An increased plasma concentration of 3-hydroxyisovaleryl carnitine (3HIA-carnitine) results from impairment in the leucine catabolic pathway at the conversion of 3-methyl-crotonyl-CoA to 3-methylglutaconyl-CoA which is caused by reduced activity of the biotin-dependent enzyme 3-...
Background Elevated plasma levels of 3-hydroxyisovaleryl-carnitine (C5OH) and impaired leucine catabolism are frequently observed in newborn screening reports, necessitating consideration of various diseases in the differential diagnosis. This study aimed to analyze different forms of C5OH and explore ...
Biomarkers such as alanine, propionylcarnitine (C3) and 3-hydroxyisovalerylcarnitine (C5-OH), which are used to diagnose biotinidase deficiency, are also present. Materials and methods In cases with partial biotinidase deficiency and normal biotinidase activity, alanine, C3 and C5-OH levels were ...