Cerebroside sulfate activator protein is a small, heat-stable protein that is exceptionally resistant to proteolytic attack. This protein is essential for the catabolism of cerebroside sulfate and several other glycosphingolipids. Protein purified from pig kidney and human urine was extensively characterized by reversed-phase liquid chromatography and electrospray mass spectrometry. These two sources revealed 20 and 18 different molecular isoforms of the protein, respectively. Plausible explanations of the structures of the majority of these isoforms can be made on the basis of accurate molecular mass assignments. The reversed-phase chromatographic and electrospray mass spectrometric properties of enzymatically deglycosylated and disulfide-reduced protein were also compared. In addition to a demonstration of the power of electrospray ionization mass spectrometry for revealing a wealth of information on protein microheterogeneity and structural detail, the results also demonstrate the utility of this technique for monitoring spontaneous chemical and enzymatically mediated changes that occur as a result of metabolic processing and protein purification.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/(SICI)1096-9888(199910)34:10<1040::AID-JMS863>3.0.CO;2-X | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Insight into adult-onset metachromatic leukodystrophy with optic atrophy: A comprehensive case report.
Radiol Case Rep
November 2023
Patan Academy of Health Science, Kathmandu, Nepal.
Article Synopsis
- Metachromatic leukodystrophy (MLD) is an autosomal recessive disorder caused by a deficiency in arylsulfatase A, leading to accumulation of cerebroside sulfate and subsequent demyelination.
- Clinical symptoms differ with age: late-infantile has rapid progression, juvenile shows slower decline, and adult-onset may present with psychiatric issues.
- Diagnosis involves enzyme testing, imaging, and other criteria, and while there's no cure for MLD, management focuses on supportive care and potential treatments like hematopoietic stem cell transplantation.
Cerebral Spinal Fluid levels of Cytokines are elevated in Patients with Metachromatic Leukodystrophy.
Sci Rep
April 2016
Division of Pediatric Blood and Marrow Transplantation, University of Minnesota, Minneapolis, Minnesota, 55455, US.
Metachromatic leukodystrophy (MLD) is a lysosomal storage disease resulting from a deficiency of arylsulfatase A causing an accumulation of cerebroside sulfate, a lipid normally abundant in myelin. Sulfatide accumulation is associated with progressive demyelination and a clinical presentation in severe disease forms that is dominated by motor manifestations. Cerebral inflammation may contribute to the pathophysiology of MLD.
View Article and Find Full Text PDFA Rare Case of Metachromatic Leukodystrophy Confirmed by Arylsulfatase A.
Mymensingh Med J
October 2015
Dr Syeda Tabassum Alam, Associate Professor, Paediatric Neurology, Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh.
Metachromatic leukodystrophy (MLD) is the rare neurometabolic disease caused by the deficiency of a lysosomal enzyme arylsulfatase A (ARSA) activity. The absence or deficiency of arylsulfatase a leads to accumulation of cerebroside sulfate within the myelinseath of the central nervous system (CNS) and the peripheral nervous system (PNS). This in turn causes the CNS and PNS to progressively deteriorate leading to both features of upper and lower motor neuron dysfunctions.
View Article and Find Full Text PDFDetermination of arylsulfatase A pseudodeficiency allele and haplotype frequency in the Tunisian population.
Neurol Sci
March 2016
Laboratoire de Génomique Biomédicale et Oncogénétique, Institut Pasteur de Tunis, BP 74, 13 Place Pasteur, 1002, Tunis, Belvédère, Tunisia.
Arylsulfatase A (ASA) is a lysosomal enzyme involved in the catabolism of cerebroside sulfate. ASA deficiency is associated with metachromatic leukodystrophy (MLD). Low ASA activities have also been reported in a more common condition with no apparent clinical consequences termed ASA pseudo-deficiency (ASA-PD) which is associated with two linked mutations in the ASA gene (c.
View Article and Find Full Text PDFA capillary electrophoresis method with dynamic pH junction stacking for the monitoring of cerebroside sulfotransferase.
J Chromatogr A
August 2015
PharmaCenter Bonn, Pharmaceutical Institute, Pharmaceutical Chemistry I, University of Bonn, An der Immenburg 4, 53121 Bonn, Germany. Electronic address:
Metachromatic leukodystrophy (MLD) is a rare and severe genetic disease. Inhibition of cerebroside sulfotransferase (CST) has been proposed as a promising new therapeutic strategy for the treatment of MLD. CST catalyzes the transfer of a sulfate group from the coenzyme 3'-phosphoadenosine-5'-phosphosulfate (PAPS) to cerebroside yielding cerebroside sulfate and adenosine-3',5'-diphosphate (PAP).
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!