10343-58-5 Purity
40%
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The metabolite 2-hydroxysebacic acid (2-HS) has been recognized as one of the most important biochemical markers of peroxisomal diseases. Stereic differences in 2-HS in urine from patients with Zellweger syndrome (ZS) were studied and compared with the enantiomeric distribution of 2-HS in urine samples from medium-chain triglyceride (MCT)-fed preterm infants.
Patients and methods
· Patients: Urine samples were obtained from 9 patients with ZS. Clinically, all had the classical features of ZS and all had elevated levels of VLCFA, abnormal plasma bile acids and decreased levels of plasmalogens in cultured skin ¢broblasts.
· Methods: (R), (S)-2-Hydroxysebacic acid dimethyl ester and (R)-3-hydroxysebacic acid dimethyl ester were synthesized in the laboratory. Hydroxydicarboxylic acids were converted to their respective methyl esters (acetyl chloridemethanol, 1:9 v/v, 10 min at 110°C) after lyophilization of the urine samples (1 ml). The methyl esters were then extracted with t-methylbutyl ether (1 ml) and concentrated to 50 ml prior to analysis.
Reliable Biomarker for Peroxisomal Disorders
I have found 2-Hydroxysebacic Acid from Alfa Chemistry to be a reliable biomarker for detecting peroxisomal disorders in children. This product has been essential in my research, specifically in identifying neonatal adrenoleukodystrophy and Zellweger syndrome through urine samples. Additionally, the ease of use and accuracy of the results have made this product invaluable in my scientific investigations. I highly recommend 2-Hydroxysebacic Acid for any researchers looking to study peroxisomal disorders.
The molecular formula is C10H18O5.
The synonyms are 2-hydroxydecanedioic acid, 103963-71-9, and decanedioic acid, 2-hydroxy-.
The molecular weight is 218.25 g/mol.
It was created on August 8, 2005.
The IUPAC name is 2-hydroxydecanedioic acid.
The InChI is "InChI=1S/C10H18O5/c11-8(10(14)15)6-4-2-1-3-5-7-9(12)13/h8,11H,1-7H2,(H,12,13)(H,14,15)".
The InChIKey is "LPIOYESQKJFWPQ-UHFFFAOYSA-N".
The canonical SMILES is "C(CCCC(C(=O)O)O)CCCC(=O)O".
The CAS number is 103963-71-9.
The melting point is 118 - 121 °C.