The protein encoded by this gene hydrolyzes cerebrosidesulfate to cerebroside and sulfate. Defects in this gene lead tometachromatic leucodystrophy (MLD), a progressive demyelinationdisease which results in a variety of neurological symptoms andultimately death. Alternatively spliced transcript variants havebeen described for this gene.
Pathway
Glycosphingolipid metabolism; Lysosome; Metabolism; Metabolism of lipids and lipoproteins; Metabolism of proteins; PTM: gamma carboxylation, hypusine formation and arylsulfatase activation; Post-translational protein modification; Sphingolipid metabolism;
Citations
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References
Comparative study of the phospha- and arsa-Wittig reaction using H-1, As-75 and O-17 NMR spectroscopy
The existence of oxaarsetanes during an arsa-Wittig reaction has been proved by H-1 and O-17 NMR spectroscopy. As-75 NMR spectra were obtained from the corresponding arsonium salts and arsane oxides. The dynamic H-1 NMR spectra of phospha- and arsaylides were compared. ((c) Wiley-VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2006).
Radioxenon production through neutron irradiation of stable xenon gas
JOURNAL OF RADIOANALYTICAL AND NUCLEAR CHEMISTRY
Authors: Haas, D. A.; Biegalski, S. R.; Biegalski, K. M. Foltz
The Spectral Deconvolution Analysis Tool (SDAT) software was developed to improve counting statistics and detection limits for nuclear explosion radionuclide measurements. SDAT utilizes spectral deconvolution spectroscopy techniques and can analyze both beta-gamma coincidence spectra for radioxenon isotopes and high-resolution HPGe spectra from aerosol monitors. The deconvolution algorithm of the SDAT requires a library of beta-gamma coincidence spectra of individual radioxenon isotopes to determine isotopic ratios in a sample. In order to get experimentally produced spectra of the individual isotopes, we have irradiated enriched samples of Xe-130, Xe-132, and Xe-134 gas with a neutron beam from the TRIGA reactor at The University of Texas. The samples were counted in an Automated Radioxenon Sampler/Analyzer (ARSA) style beta-gamma coincidence detector. The spectra produced show that this method of radioxenon production yields samples with very high purity of the individual isotopes for Xe-131m and Xe-135 and a sample with a substantial Xe-133m to Xe-133 ratio.
COA
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