Steryl-Sulfatase
Iduronate Sulfatase
Chondro-4-Sulfatase
Arylsulfatases
N-Acetylgalactosamine-4-Sulfatase
An arylsulfatase that catalyzes the hydrolysis of the 4-sulfate groups of the N-acetyl-D-galactosamine 4-sulfate units of chondroitin sulfate and dermatan sulfate. A deficiency of this enzyme is responsible for the inherited lysosomal disease, Maroteaux-Lamy syndrome (MUCOPOLYSACCHARIDOSIS VI). EC 3.1.6.12.
Chondroitinsulfatases
Mucopolysaccharidosis II
Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.
Multiple Sulfatase Deficiency Disease
An inherited metabolic disorder characterized by the intralysosomal accumulation of sulfur-containing lipids (sulfatides) and MUCOPOLYSACCHARIDES. Excess levels of both substrates are present in urine. This is a disorder of multiple sulfatase (arylsulfatases A, B, and C) deficiency which is caused by the mutation of sulfatase-modifying factor-1. Neurological deterioration is rapid.
Ichthyosis, X-Linked
Cerebroside-Sulfatase
An enzyme that catalyzes the hydrolysis of cerebroside 3-sulfate (sulfatide) to yield a cerebroside and inorganic sulfate. A marked deficiency of arylsulfatase A, which is considered the heat-labile component of cerebroside sulfatase, has been demonstrated in all forms of metachromatic leukodystrophy (LEUKODYSTROPHY, METACHROMATIC). EC 3.1.6.8.
Mucopolysaccharidosis IV
Ichthyosis
Sulfotransferases
Mucopolysaccharidosis VI
Leukodystrophy, Metachromatic
An autosomal recessive metabolic disease caused by a deficiency of CEREBROSIDE-SULFATASE leading to intralysosomal accumulation of cerebroside sulfate (SULFOGLYCOSPHINGOLIPIDS) in the nervous system and other organs. Pathological features include diffuse demyelination, and metachromatically-staining granules in many cell types such as the GLIAL CELLS. There are several allelic and nonallelic forms with a variety of neurological symptoms.
Sphingolipidoses
A group of inherited metabolic disorders characterized by the intralysosomal accumulation of SPHINGOLIPIDS primarily in the CENTRAL NERVOUS SYSTEM and to a variable degree in the visceral organs. They are classified by the enzyme defect in the degradation pathway and the substrate accumulation (or storage). Clinical features vary in subtypes but neurodegeneration is a common sign.
Estrone
An aromatized C18 steroid with a 3-hydroxyl group and a 17-ketone, a major mammalian estrogen. It is converted from ANDROSTENEDIONE directly, or from TESTOSTERONE via ESTRADIOL. In humans, it is produced primarily by the cyclic ovaries, PLACENTA, and the ADIPOSE TISSUE of men and postmenopausal women.
Mucopolysaccharidoses
Ichthyosis Vulgaris
17-Hydroxysteroid Dehydrogenases
Lysosomal Storage Diseases
Mucopolysaccharidosis III
Coumarins
X Chromosome
Dehydroepiandrosterone Sulfate
Dydrogesterone
Lysosomes
A class of morphologically heterogeneous cytoplasmic particles in animal and plant tissues characterized by their content of hydrolytic enzymes and the structure-linked latency of these enzymes. The intracellular functions of lysosomes depend on their lytic potential. The single unit membrane of the lysosome acts as a barrier between the enzymes enclosed in the lysosome and the external substrate. The activity of the enzymes contained in lysosomes is limited or nil unless the vesicle in which they are enclosed is ruptured. Such rupture is supposed to be under metabolic (hormonal) control. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)
Peptococcus
Chondroitinases and Chondroitin Lyases
Glycosaminoglycans
Enzyme Replacement Therapy
Chondrodysplasia Punctata
A heterogeneous group of bone dysplasias, the common character of which is stippling of the epiphyses in infancy. The group includes a severe autosomal recessive form (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC), an autosomal dominant form (Conradi-Hunermann syndrome), and a milder X-linked form. Metabolic defects associated with impaired peroxisomes are present only in the rhizomelic form.
Azasteroids
Aromatase
An enzyme that catalyzes the desaturation (aromatization) of the ring A of C19 androgens and converts them to C18 estrogens. In this process, the 19-methyl is removed. This enzyme is membrane-bound, located in the endoplasmic reticulum of estrogen-producing cells of ovaries, placenta, testes, adipose, and brain tissues. Aromatase is encoded by the CYP19 gene, and functions in complex with NADPH-FERRIHEMOPROTEIN REDUCTASE in the cytochrome P-450 system.
Prevotella
A genus of gram-negative, anaerobic, nonsporeforming, nonmotile rods. Organisms of this genus had originally been classified as members of the BACTEROIDES genus but overwhelming biochemical and chemical findings in 1990 indicated the need to separate them from other Bacteroides species, and hence, this new genus was established.
Fibroblasts
Sex Chromosome Aberrations
Norpregnadienes
Dermatan Sulfate
Hydrolases
Any member of the class of enzymes that catalyze the cleavage of the substrate and the addition of water to the resulting molecules, e.g., ESTERASES, glycosidases (GLYCOSIDE HYDROLASES), lipases, NUCLEOTIDASES, peptidases (PEPTIDE HYDROLASES), and phosphatases (PHOSPHORIC MONOESTER HYDROLASES). EC 3.
Dehydroepiandrosterone
A major C19 steroid produced by the ADRENAL CORTEX. It is also produced in small quantities in the TESTIS and the OVARY. Dehydroepiandrosterone (DHEA) can be converted to TESTOSTERONE; ANDROSTENEDIONE; ESTRADIOL; and ESTRONE. Most of DHEA is sulfated (DEHYDROEPIANDROSTERONE SULFATE) before secretion.
Chondroitin Lyases
Enzymes which catalyze the elimination of delta-4,5-D-glucuronate residues from polysaccharides containing 1,4-beta-hexosaminyl and 1,3-beta-D-glucuronosyl or 1,3-alpha-L-iduronosyl linkages thereby bringing about depolymerization. EC 4.2.2.4 acts on chondroitin sulfate A and C as well as on dermatan sulfate and slowly on hyaluronate. EC 4.2.2.5 acts on chondroitin sulfate A and C.
Neoplasms, Hormone-Dependent
Chondroitin Sulfates
Derivatives of chondroitin which have a sulfate moiety esterified to the galactosamine moiety of chondroitin. Chondroitin sulfate A, or chondroitin 4-sulfate, and chondroitin sulfate C, or chondroitin 6-sulfate, have the sulfate esterified in the 4- and 6-positions, respectively. Chondroitin sulfate B (beta heparin; DERMATAN SULFATE) is a misnomer and this compound is not a true chondroitin sulfate.
Hybrid Cells
Tyramine
An indirect sympathomimetic. Tyramine does not directly activate adrenergic receptors, but it can serve as a substrate for adrenergic uptake systems and monoamine oxidase so it prolongs the actions of adrenergic transmitters. It also provokes transmitter release from adrenergic terminals. Tyramine may be a neurotransmitter in some invertebrate nervous systems.
Megestrol
Sex Chromosomes
The homologous chromosomes that are dissimilar in the heterogametic sex. There are the X CHROMOSOME, the Y CHROMOSOME, and the W, Z chromosomes (in animals in which the female is the heterogametic sex (the silkworm moth Bombyx mori, for example)). In such cases the W chromosome is the female-determining and the male is ZZ. (From King & Stansfield, A Dictionary of Genetics, 4th ed)
Sulfuric Acids
Heparitin Sulfate
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Placenta
A highly vascularized mammalian fetal-maternal organ and major site of transport of oxygen, nutrients, and fetal waste products. It includes a fetal portion (CHORIONIC VILLI) derived from TROPHOBLASTS and a maternal portion (DECIDUA) derived from the uterine ENDOMETRIUM. The placenta produces an array of steroid, protein and peptide hormones (PLACENTAL HORMONES).
Dosage Compensation, Genetic
Genetic mechanisms that allow GENES to be expressed at a similar level irrespective of their GENE DOSAGE. This term is usually used in discussing genes that lie on the SEX CHROMOSOMES. Because the sex chromosomes are only partially homologous, there is a different copy number, i.e., dosage, of these genes in males vs. females. In DROSOPHILA, dosage compensation is accomplished by hypertranscription of genes located on the X CHROMOSOME. In mammals, dosage compensation of X chromosome genes is accomplished by random X CHROMOSOME INACTIVATION of one of the two X chromosomes in the female.
Clinical Enzyme Tests
Bacteroides
Pregnenolone
Food Additives
Substances which are of little or no nutritive value, but are used in the processing or storage of foods or animal feed, especially in the developed countries; includes ANTIOXIDANTS; FOOD PRESERVATIVES; FOOD COLORING AGENTS; FLAVORING AGENTS; ANTI-INFECTIVE AGENTS (both plain and LOCAL); VEHICLES; EXCIPIENTS and other similarly used substances. Many of the same substances are PHARMACEUTIC AIDS when added to pharmaceuticals rather than to foods.
Estrogens
Compounds that interact with ESTROGEN RECEPTORS in target tissues to bring about the effects similar to those of ESTRADIOL. Estrogens stimulate the female reproductive organs, and the development of secondary female SEX CHARACTERISTICS. Estrogenic chemicals include natural, synthetic, steroidal, or non-steroidal compounds.
Hexosaminidases
Substrate Specificity
Glycine
Alanine
Cells, Cultured
Catalytic Domain
Estradiol
Keratan Sulfate
A sulfated mucopolysaccharide initially isolated from bovine cornea. At least two types are known. Type I, found mostly in the cornea, contains D-galactose and D-glucosamine-6-O-sulfate as the repeating unit; type II, found in skeletal tissues, contains D-galactose and D-galactosamine-6-O-sulfate as the repeating unit.
Y Chromosome
Genetic Linkage
Metabolism, Inborn Errors
Protein Processing, Post-Translational
Any of various enzymatically catalyzed post-translational modifications of PEPTIDES or PROTEINS in the cell of origin. These modifications include carboxylation; HYDROXYLATION; ACETYLATION; PHOSPHORYLATION; METHYLATION; GLYCOSYLATION; ubiquitination; oxidation; proteolysis; and crosslinking and result in changes in molecular weight and electrophoretic motility.
Mutation
Heparan Sulfate Proteoglycans
Ubiquitous macromolecules associated with the cell surface and extracellular matrix of a wide range of cells of vertebrate and invertebrate tissues. They are essential cofactors in cell-matrix adhesion processes, in cell-cell recognition systems, and in receptor-growth factor interactions. (From Cancer Metastasis Rev 1996; 15(2): 177-86; Hepatology 1996; 24(3): 524-32)
Mosaicism
Amino Acid Sequence
Chromatography, High Pressure Liquid
Base Sequence
Point Mutation
Phenotype
RNA, Messenger
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Pseudomonas
Skin
Microsomes
Artifactual vesicles formed from the endoplasmic reticulum when cells are disrupted. They are isolated by differential centrifugation and are composed of three structural features: rough vesicles, smooth vesicles, and ribosomes. Numerous enzyme activities are associated with the microsomal fraction. (Glick, Glossary of Biochemistry and Molecular Biology, 1990; from Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)
Carcinoma, Endometrioid
Enzyme Inhibitors
Sequence Homology, Amino Acid
Hydrogen-Ion Concentration
Heparin
A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts.
Leukocytes
Gene Expression Regulation, Enzymologic
Serine
Carrageenan
Genes
Gene Deletion
Isoelectric Focusing
Chromosome Mapping
Oligosaccharides
Liver
Heterozygote
Report of a mucopolysaccharidosis occurring in Australian aborigines. (1/74)
The first 2 reported cases of a mucopolysaccharidosis occurring in an Australian aboriginal family are presented. Though these children had the characteristic morphological features of the Hurler syndrome, enzyme assay of cultured fibroblasts showed normal levels of alpha-L-iduronidase and decreased activity of arylsulphatase B. Thus, they represented the Hurler syndrome clinically, while they had the enzyme defect of the Maroteaux-Lamy syndrome, and they may represent a new severe form of the Maroteaux-Lamy syndrome. The parents of these children were first cousins. Though the children were not full blood aborigines, examination of the pedigree indicates that the gene originated in the common aboriginal family. (+info)Microanalysis of enzyme digests of hyaluronan and chondroitin/dermatan sulfate by fluorophore-assisted carbohydrate electrophoresis (FACE). (2/74)
Hyaluronan and chondroitin/dermatan sulfate are glycosaminoglycans that play major roles in the biomechanical properties of a wide variety of tissues, including cartilage. A chondroitin/dermatan sulfate chain can be divided into three regions: (1) a single linkage region oligosaccharide, through which the chain is attached to its proteoglycan core protein, (2) numerous internal repeat disaccharides, which comprise the bulk of the chain, and (3) a single nonreducing terminal saccharide structure. Each of these regions of a chondroitin/dermatan sulfate chain has its own level of microheterogeneity of structure, which varies with proteoglycan class, tissue source, species, and pathology. We have developed rapid, simple, and sensitive protocols for detection, characterization and quantitation of the saccharide structures from the internal disaccharide and nonreducing terminal regions of hyaluronan and chondroitin/dermatan sulfate chains. These protocols rely on the generation of saccharide structures with free reducing groups by specific enzymatic treatments (hyaluronidase/chondroitinase) which are then quantitatively tagged though their free reducing groups with the fluorescent reporter, 2-aminoacridone. These saccharide structures are further characterized by modification through additional enzymatic (sulfatase) or chemical (mercuric ion) treatments. After separation by fluorophore-assisted carbohydrate electrophoresis, the relative fluorescence in each band is quantitated with a cooled, charge-coupled device camera for analysis. Specifically, the digestion products identified are (1) unsaturated internal Deltadisaccharides including DeltaDiHA, DeltaDi0S, DeltaDi2S, DeltaDi4S, DeltaDi6S, DeltaDi2,4S, DeltaDi2,6S, DeltaDi4,6S, and DeltaDi2,4,6S; (2) saturated nonreducing terminal disaccharides including DiHA, Di0S, Di4S and Di6S; and (3) nonreducing terminal hexosamines including glcNAc, galNAc, 4S-galNAc, 6S-galNAc, and 4, 6S-galNAc. (+info)Slow reacting substance as a preformed mediator from human lung. (3/74)
Homogenates from human lung contained a preformed slow reacting substance (pSRS). The pattern of contraction on the guinea-pig ileum by pSRS was indistinguishable from that of SRS-A. The activity of pSRS could not be attributed to the presence of K+, Na+, Ca2+ and Mg2+ ions, or any prostaglandin including PGF2 or its 15-oxo derivative. As with SRS-A, pSRS could be absorbed onto Amberlite XAD-2 and silicic acid. Both were eluted from the former with 80 per cent ethanol and from the latter with a mixture of ethanol, ammonia and water. Both pSRS and SRS-A were resistant to the action of NaOH whereas their activities were destroyed by boiling in HCl. Arylsulphatase II B destroyed the activities of both pSRS and SRS-A. An antagonist of SRS-A, FPL55712, inhibited the action of pSRS at comparable concentrations to that of SRS-A. These experiments suggest that pSRS and SRS-A are identical. Thus SRS joins histamine and ECF-A as a preformed mediator. Although SRS was present in a preformed state the amount of material extractable was more than doubled by the anaphylactic reaction. The extraction of slow reacting substance from human lung without apparent requirement for antigen or antibody points to a possible role of this mediator in inflammatory reactions evoked by mechanisms independent of IgE and other tissue-sensitizing antibodies. (+info)Correction of human mucopolysaccharidosis type-VI fibroblasts with recombinant N-acetylgalactosamine-4-sulphatase. (4/74)
A full-length human N-acetylgalactosamine-4-sulphatase (4-sulphatase) cDNA clone was constructed and expressed in CHO-DK1 cells under the transcriptional control of the Rous sarcoma virus long terminal repeat. A clonal cell line expressing high activities of human 4-sulphatase was isolated. The maturation and processing of the human enzyme in this transfected CHO cell line showed it to be identical with that seen in normal human skin fibroblasts. The high-uptake precursor form of the recombinant enzyme was purified from the medium of the transfected cells treated with NH4Cl and was shown to be efficiently endocytosed by control fibroblasts and by fibroblasts from a mucopolysaccharidosis type-VI (MPS VI) patient. Enzyme uptake was inhibitable by mannose 6-phosphate. After uptake, the enzyme was processed normally in both normal and MPS VI fibroblasts and was shown both to correct the enzymic defect and to initiate degradation of [35S]sulphated dermatan sulphate in MPS VI fibroblasts. The stabilities of the recombinant enzyme and enzyme from human fibroblasts appeared to be similar after uptake. However, endocytosed enzyme has a significantly shorter half-life than endogenous human enzyme. The purified precursor 4-sulphatase had a similar pH optimum and catalytic parameters to the mature form of 4-sulphatase isolated from human liver. (+info)Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAs. (5/74)
Multiple sulfatase deficiency (MSD) is an inherited lysosomal storage disease characterized by the deficiency of at least seven sulfatases. The basic defect in MSD is thought to be in a post-translational modification common to all sulfatases. In accordance with this concept, RNAs of normal size and amount were detected in MSD fibroblasts for three sulfatases tested. cDNAs encoding arylsulfatase A, arylsulfatase B, or steroid sulfatase were introduced into MSD fibroblasts and fibroblasts with a single sulfatase deficiency by retroviral gene transfer. Infected fibroblasts overexpressed the respective sulfatase polypeptides. While in single-sulfatase-deficiency fibroblasts a concomitant increase of sulfatase activities was observed, MSD fibroblasts expressed sulfatase polypeptides with a severely diminished catalytic activity. From these results we conclude that the mutation in MSD severely decreases the capacity of a co- or post-translational process that renders sulfatases enzymatically active or prevents their premature inactivation. (+info)Localization of arylsulphatase A and B activities in rat kidney. (6/74)
Very high arylsulphatase activity has been detected in rat kidney. It is the highest in renal cortex (19 U/g tissue), 3-30 times higher than in other rat organs. Histochemically, arylsulphatase B (N-acetylgalactosamine-4-sulphate sulphatase) activity is localized in large lysosomes of proximal convoluted tubules, where it accounts for over 90% of total arylsulphatase activity. This suggests that the enzyme plays an important role in the degradation of endocytosed sulphated oligosaccharides. (+info)Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). An intermediate clinical phenotype caused by substitution of valine for glycine at position 137 of arylsulfatase B. (7/74)
The Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) is a lysosomal storage disease with autosomal recessive inheritance caused by deficiency of the enzyme arylsulfatase B. Severe, intermediate, and mild forms of the disease have been described. The molecular correlate of the clinical heterogeneity is not known at present. To identify the molecular defect in a patient with the intermediate form of the disease, arylsulfatase B mRNA from his fibroblasts was reverse-transcribed, amplified by the polymerase chain reaction, and subcloned. Three point mutations were detected by DNA sequence analysis, two of which, a silent A to G transition at nucleotide 1191 and a G to A transition at nucleotide 1126 resulting in a methionine for valine 376 substitution, were polymorphisms. A G to T transversion at nucleotide 410 causing a valine for glycine 137 substitution (G137V) was identified as the mutation underlying the Maroteaux-Lamy phenotype of the patient, who was homozygous for the allele. The kinetic parameters of the mutant arylsulfatase B enzyme toward a radiolabeled trisaccharide substrate were normal excluding an alteration of the active site. The G137V mutation did not affect the synthesis but severely reduced the stability of the arylsulfatase B precursor. While the wild type precursor is converted by limited proteolysis in late endosomes or lysosomes to a mature form, the majority of the mutant precursor was degraded presumably in a compartment proximal to the trans Golgi network and only a small amount escaped to the lysosomes accounting for the low residual enzyme activity in fibroblasts of a patient with the juvenile form of the disease. (+info)Evidence for differential regulation of genes in the chondroitin sulfate utilization pathway of Bacteroides thetaiotaomicron. (8/74)
Expression of the chondroitin sulfate utilization (csu) genes of Bacterioides thetaiotaomicron is regulated by chondroitin sulfate. We have now found, however, that the csu genes are not all regulated in the same way. In particular, the gene encoding beta-glucuronidase (csuE) is expressed under two different conditions that do not lead to expression of other csu genes. (+info)
Arylsulfatase B
ARSB - Performance: Arylsulfatase B, Fibroblasts
anti-ARSB antibody [N3C3] | GeneTex
Open-Label Study of Efficacy and Safety of Recombinant Human N-acetylgalactosamine 4-sulfatase in Patients With MPS VI - Full...
Decline in arylsulfatase B leads to increased invasiveness of melanoma cells<...
MPS VI - mps-europe.org
Myelopathy Associated With Maroteaux-Lamy Syndrome | JAMA Neurology | The JAMA Network
Oral and systemic manifestations of mucopolysaccharidosis type VI: A report of seven cases
Mucopolysaccharidosis VI
- Syndrome, Maroteaux-Lamy
Summary Report | CureHunter
Relative distribution of arylsulphatases A and B in rat liver parenchymal and other cells | Biochemical Journal
Maroteaux-Lamy syndrome (MPSVI) - Greenwood Genetic Center
Mucopolysaccharidosis VI Symptoms, Diagnosis, Treatments and Causes - RightDiagnosis.com
Biomarker for Maroteaux-Lamy Disease - Full Text View - ClinicalTrials.gov
PRIME® Clinical Case Study: Skeletal Abnormalities Lead to Diagnosis of MPS VI
Transcultural Understanding of a Hereditary Disorder: Mucopolysaccharidosis VI in a Vietnamese Family<...
Arylsulfatases
Summary Report | CureHunter
Maroteaux-Lamy syndrome
January 2009 - Volume 32 - Issue 1 : Journal of Infusion Nursing
Mucopolysaccharidosis VI
Cat combination Birman
Targeting Chondroitin Sulfate Glycosaminoglycans to Treat Cardiac Fibrosis in Pathological Remodeling | Circulation
Induction of chondro-, osteo- and adipogenesis in embryonic stem cells by bone morphogenetic protein-2: Effect of cofactors on...
Sulfatase Modifying Factor 2/SUMF2 Research Products: Novus Biologicals
Anti Human Sulfatase 2 (C-Terminal) Antibody, clone 2B4 | Bio-Rad
Anti Human Sulfatase 2 (C-Terminal) Antibody, clone 2B4 | Bio-Rad Antibodies (formerly AbD Serotec)
Plus it
Anti-Iduronate 2 sulfatase antibody (ab85701) Protocols
Sulfatase 2抗体|Abcam中国|Anti-Sulfatase 2抗体(ab101057)
Molecular and functional analysis of SUMF1 mutations in multiple sulfatase deficiency. - Physiology, Anatomy and Genetics
Molecular and functional analysis of SUMF1 mutations in multiple sulfatase deficiency
Project
MSD Research in progress | MSD Action Foundation
What rhymes with n-acetylgalactosamine-4-sulfatase?
anti-Arylsulfatase A antibody [N2C2], Internal | GeneTex
Management of Metabolic Bone Disease in Children - The ISPN Guide to Pediatric Neurosurgery
Galactosamine-6 sulfatase - Wikipedia
1y1e - Proteopedia, life in 3D
TEDE: Relato de caso de um paciente com mucopolissacaridose tipo VI (Síndrome de Maroteaux-Lamy)
Arylsulfatase E - definition of Arylsulfatase E by The Free Dictionary
Isolation and characterization of the pig endometrial arylsulphatase A. | Biochemical Journal
ARSH Polyclonal Antibody, FITC Conjugated | EpiGentek
Electrophoretic Characterization of A and B Isoenzymes of Arylsulfatase | Biochemical Society Transactions | Portland Press
iduronate 2 sulfatase Antibody 17140-1-AP | Proteintech
Arcoxia «MSD» - Felleskatalogen
Evaluation of Aminoglycoside and Non-Aminoglycoside Compounds for Stop-Codon Readthrough Therapy in Four Lysosomal Storage...
Arylsulfatase A pseudodeficiency - SNPedia
Global Rx Pharmacy: Placental sulfatase and prednisolone overnight shipping!
Steroid sulfatase抗体|Abcam中国|Anti-Steroid sulfatase抗体(ab62219)
Glusulase (contains both β-Glucuronidase and β-Glucuronide Sulfatase), 10mL | PerkinElmer
Myc-DDK-tagged ORF clone of Homo sapiens sulfatase 1 (SULF1), transcript variant 1 as transfection-ready DNA - RC226222 - 10 µg...
Arylsulfatase A, Leukocytes
Dried Banana Powder,Cameroon Dried Banana Powder price supplier - 21food
Chondro-4-sulfatase
In enzymology, a chondro-4-sulfatase (EC 3.1.6.9) is an enzyme that catalyzes the chemical reaction 4-deoxy-beta-D-gluc-4- ... The systematic name of this enzyme class is 4-deoxy-beta-D-gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine-4-su lfate 4- ... This enzyme is also called chondroitin-4-sulfatase. Held E, Buddecke E (1967). "[Studies on the chemistry of the arterial wall ... Demonstration, purification and properties of a chondroitin-4-sulfatase from bovine aorta]". Hoppe-Seyler's Z. Physiol. Chem. ...
List of MeSH codes (D08)
... cerebroside-sulfatase MeSH D08.811.277.352.827.070.625 - steryl-sulfatase MeSH D08.811.277.352.827.180 - chondroitinases and ... chondro-4-sulfatase MeSH D08.811.277.352.827.500 - iduronate sulfatase MeSH D08.811.277.352.897 - thiolester hydrolases MeSH ... sulfatases MeSH D08.811.277.352.827.070 - arylsulfatases MeSH D08.811.277.352.827.070.060 - n-acetylgalactosamine-4-sulfatase ... 4-beta-glucosidase MeSH D08.811.277.450.420.200.600 - glucan endo-1,3-beta-d-glucosidase MeSH D08.811.277.450.420.375 - glucan ...
Chondro-6-sulfatase
In enzymology, a chondro-6-sulfatase (EC 3.1.6.10) is an enzyme that catalyzes the chemical reaction 4-deoxy-beta-D-gluc-4- ... The systematic name of this enzyme class is 4-deoxy-beta-D-gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine-6-su lfate 6- ... 4-deoxy-beta-D-gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine + sulfate The 3 substrates of this enzyme are 4-deoxy-beta-D- ... gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine, 6-sulfate, and H2O, whereas its two products are 4-deoxy-beta-D-gluc-4- ...
List of EC numbers (EC 3)
... choline-sulfatase EC 3.1.6.7: cellulose-polysulfatase EC 3.1.6.8: cerebroside-sulfatase EC 3.1.6.9: chondro-4-sulfatase EC 3.1. ... 6.10: chondro-6-sulfatase EC 3.1.6.11: disulfoglucosamine-6-sulfatase EC 3.1.6.12: N-acetylgalactosamine-4-sulfatase EC 3.1. ... N-sulfoglucosamine-3-sulfatase EC 3.1.6.16: monomethyl-sulfatase EC 3.1.6.17: D-lactate-2-sulfatase EC 3.1.6.18: glucuronate-2- ... steryl-sulfatase EC 3.1.6.3: glycosulfatase EC 3.1.6.4: N-acetylgalactosamine-6-sulfatase EC 3.1.6.5: deleted EC 3.1.6.6: ...
Chondro-4-sulfatase - Wikipedia
In enzymology, a chondro-4-sulfatase (EC 3.1.6.9) is an enzyme that catalyzes the chemical reaction 4-deoxy-beta-D-gluc-4- ... The systematic name of this enzyme class is 4-deoxy-beta-D-gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine-4-su lfate 4- ... This enzyme is also called chondroitin-4-sulfatase. Held E, Buddecke E (1967). "[Studies on the chemistry of the arterial wall ... Demonstration, purification and properties of a chondroitin-4-sulfatase from bovine aorta]". Hoppe-Seylers Z. Physiol. Chem. ...
Enzyme Explorer Assay Library | Sigma-Aldrich
Chondroitin-4-sulfation negatively regulates axonal guidance and growth | Journal of Cell Science
Chondro-4-sulfatase (Seikagaku) digestion was carried out with 8 mU of the enzyme in 0.1 M ammonium acetate buffer (pH 7.0) at ... Axons were repelled by sulfatase-treated CSPGs at comparable levels to non-treated CSPGs (data not shown). CS-A was then ... To exclude the possibility that the presence of cABC in the chondro-4-sulfatase preparation was responsible for the drastic ... Note that chondroitin sulfate disaccharides are good substrates for chondro-4-sulfatase, but intact CSPGs are not (Yamagata et ...
Adelaide Research & Scholarship: Identification, expression, and biochemical characterization of N-acetylgalactosamine-4...
CHO Cells; Animals; Humans; Mucopolysaccharidosis VI; Chondro-4-Sulfatase; Recombinant Proteins; DNA, Complementary; DNA ... Identification, expression, and biochemical characterization of N-acetylgalactosamine-4-sulfatase mutations and relationship ... characterized by the defective degradation of dermatan sulfate due to the deficiency of N-acetylgalactosamine-4-sulfatase (4S ...
Code System Concept
List of MeSH codes (D08) - Wikipedia
... cerebroside-sulfatase MeSH D08.811.277.352.827.070.625 - steryl-sulfatase MeSH D08.811.277.352.827.180 - chondroitinases and ... chondro-4-sulfatase MeSH D08.811.277.352.827.500 - iduronate sulfatase MeSH D08.811.277.352.897 - thiolester hydrolases MeSH ... sulfatases MeSH D08.811.277.352.827.070 - arylsulfatases MeSH D08.811.277.352.827.070.060 - n-acetylgalactosamine-4-sulfatase ... 4-beta-glucosidase MeSH D08.811.277.450.420.200.600 - glucan endo-1,3-beta-d-glucosidase MeSH D08.811.277.450.420.375 - glucan ...
Metachromatic leukodystrophy. Ultrastructural and enzymatic study of a case of variant O form | Meta
ENZYME: 3.1.-.
Choline-sulfatase 3.1.6.7 Cellulose-polysulfatase 3.1.6.8 Cerebroside-sulfatase 3.1.6.9 Chondro-4-sulfatase 3.1.6.10 Chondro-6- ... N-sulfoglucosamine-3-sulfatase 3.1.6.16 Monomethyl-sulfatase 3.1.6.17 D-lactate-2-sulfatase 3.1.6.18 Glucuronate-2-sulfatase ... Steryl-sulfatase 3.1.6.3 Glycosulfatase 3.1.6.4 N-acetylgalactosamine-6-sulfatase 3.1.6.5 Deleted entry 3.1.6.6 ... sulfatase 3.1.6.11 Disulfoglucosamine-6-sulfatase 3.1.6.12 N-acetylgalactosamine-4-sulfatase 3.1.6.13 Iduronate-2-sulfatase 3.1 ...
List of EC numbers (EC 3)
EC 3.1.6.10: chondro-6-sulfatase * EC 3.1.6.11: disulfoglucosamine-6-sulfatase ... EC 3.1.4: Phosphoric Diester Hydrolases. * EC 3.1.4.1: phosphodiesterase I * EC 3.1.4.2: glycerophosphocholine ... EC 3.1.3.66: phosphatidylinositol-3,4-bisphosphate 4-phosphatase * EC 3.1.3.67: phosphatidylinositol-3,4,5-trisphosphate 3- ... EC 3.2.1.141: [[4-a-D-((1(arrow to right)4)-a-D-glucano}trehalose trehalohydrolase]] ...
Lysosomal hydrolases of the epidermis. 2. Ester hydrolases. | Microbiology Department at UMass Amherst
Search | Global Index Medicus
Sulfatases that catalyze the desulfation of different sulfoconjugates are known to be deficient in a number of genetic storage ... Acetylglucosaminidase/pharmacology , Animals , Brain/enzymology , Chondro-4-Sulfatase/immunology , Immunochemistry , Macaca ... The bound enzymes could be eluted either at an acid pH of 4·5 or by mannose 6-phosphate but not by a number of other sugars ... radiata , Mannosyl-Glycoprotein Endo-beta-N-Acetylglucosaminidase , Molecular Weight , Phosphorylation , Sulfatases/isolation ...
Code System Concept
Chondro-6-sulfatase (substance) {90698004 , SNOMED-CT } Glycosulfatase (substance) {14543000 , SNOMED-CT } Heparan N-sulfatase ... N-Sulfoglucosamine-6-sulfatase (substance) {39830000 , SNOMED-CT } Placental sulfatase (substance) {259328002 , SNOMED-CT } ... N-Acetylgalactosamine-6-sulfatase (substance) {76770006 , SNOMED-CT } N-Acetylglucosamine-6-sulfatase (substance) {15145009 , ... Sulfatase (substance) {175067005 , SNOMED-CT } Parent/Child (Relationship Type) Arylsulfatase (substance) {304282007 , SNOMED- ...
Morquio Syndrome (Mucopolysaccharidosis Type IV): Background, Pathophysiology, Epidemiology
The multiple sulfatase deficiency gene encodes an essential and limiting factor for the activity of sulfatases. Cell. 2003 May ... The classics: Chondro-osteo-dystrophy. roentgenographic and clinical features of a child with dislocation of vertebrae, James F ... Murine model (Galns(tm(C76S)slu)) of MPS IVA with missense mutation at the active site cysteine conserved among sulfatase ... Murine model (Galns(tm(C76S)slu)) of MPS IVA with missense mutation at the active site cysteine conserved among sulfatase ...
Closed Meningo(encephalo)cele: A New Feature in Hunter Syndrome | American Journal of Neuroradiology
Multiple sulfatase deficiency was excluded by detection of normal activity of other lysosomial sulfatases. The phenotype was ... Areas of focal brain parenchymal and/or meningeal herniation at the level of chondro-/dermatocranium were recorded and the ... ERT with recombinant human iduronate-2-sulfatase (idursulfase; Elaprase, Shire Human Genetic Therapies, Cambridge, ... is a rare X-linked recessive disease caused by mutations of the gene encoding for iduronate-2-sulfatase, an enzyme involved in ...
Desulfation of Heparan Sulfate by Sulf1 and Sulf2 Is Required for Corticospinal Tract Formation | Scientific Reports
This classical view was revised by the discovery of the endosulfatases, Sulfatase 1 (Sulf1) and Sulfatase 2 (Sulf2), which ... and an Unsaturated Chondro-Disaccharide Kit (C-Kit) (Seikagaku). The chromatogram was analyzed using Empower software (Waters ... Secreted sulfatases Sulf1 and Sulf2 have overlapping yet essential roles in mouse neonatal survival. PLoS One 2, e575 (2007). ... Pictures (f 1 -m 1 ), (f 2 -m 2 ), (f 3 -m 3 ), and (f 4 -i 4 ) show the boxed regions with the corresponding numbers in the ...
The Quest for Height: Grow Taller | Increase Height | Bone Size: May 2011
... is exerted by sulfatases that are activated by another enzyme, Sulfatase-Modifying Factor 1 (SUMF1), whose inactivation in ... "mice conditionally deleted for FGFR1 in osteo-chondro-progenitor cells display an increased hypertrophic zone size, probably ... These enzymes are substrates of Sumf1 whose only known function is to activate sulfatases. Proteoglycan desulfation regulates ... "Sumf1, and proteoglycan desulfation, influence FGF signaling in chondrocytes during skeletal development.Sulfatases catalyze ...
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Chondro-osteo-dystrophy: Mitochondrial and whole helicases of ear with wave of glycans, Am degree Surg, 7, 1929, 404-410). ... MPSIVA is divided by a replication in N-acetylgalactosamine 6-sulfatase( GALNS; MIM:612222) which well is cytoskeletal cells of ... The vivo ZP glucose adds associated of 4 operators listed as ZP1, ZP2, ZP3 and ZP4. only, the download of the regulation to ... Winston Churchill, and a download The Hunt for of 3,4-bisphosphate mitochondrial Wayne Gretzky by Andy Warhol. form xanthine, ...
Antirheumatic drug response signatures in human chondrocytes: potential molecular targets to stimulate cartilage regeneration |...
CXCR-4, IL-1RN, IL-6/8, MMP-10/12, and TLR-2) and novel genes (ADORA2A, BCL2-A1, CTGF, CXCR-7, CYR-61, HSD11B-1, IL-23A, MARCKS ... steroid sulfatase (STS, Hs00165853_m1), and glyceraldehyde 3-phosphate dehydrogenase (GAPDH, Hs99999905_m1). The endogenous ... cartilage destruction in RA and during antirheumatic therapy and may contribute to the development of novel therapeutic chondro ... CXCR-4, IL-1RN, IL-6/8, MMP-10/12, and TLR-2) and new genes (ADORA2A, BCL2-A1, CTGF, CXCR-7, CYR-61, HSD11B-1, IL-23A, MARCKS, ...
JCI -
mTORC1 hyperactivation arrests bone growth in lysosomal storage disorders by suppressing autophagy
... due to mutations in the lysosomal sulfatase arylsulfatase B (Arsb-/-) (Supplemental Figure 2A), and in chondrocyte precursor ... is defined as the region beginning with the consistent cylindrical cellular profile and extending to the metaphyseal chondro- ... Furthermore, Gusb-/- Rpt+/- mice showed an almost normal level of collagen in the femoral growth plates (Figure 4, A-C). ... Figure 4. Normalization of mTORC1 signaling restores collagen trafficking and rescues bone phenotype in MPS VII mice. (A) ...
Osteochondrosis disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials
Randomised Multi-Center Study: Microfracturing With Chondro-Gide® for the Treatment of Isolated Cartilage Defects in the Knee ... Galactosamine (N-Acetyl)-6-Sulfatase. Protein Coding. 5.99. DISEASES inferred 15 20. COL9A1 ... 4. epiphyseal dysplasia, multiple, 1 34.0. COL9A3 COL9A2 COL9A1 5. epiphyseal dysplasia, multiple, 3 34.0. COL9A3 COL9A2 COL9A1 ... 4. collagen-containing extracellular matrix. GO:0062023 9.8. TGFB1 COL9A3 COL9A2 COL9A1 COL2A1 COL24A1 ...
BRENDA - Information on EC 3.1.6.9 - chondro-4-sulfatase
An upstream insertion mutant that has no detectable chondro-4-sulfatase activity is still able to grow on chondroitin sulfate, ... An upstream insertion mutant that has no detectable chondro-4-sulfatase activity is still able to grow on chondroitin sulfate, ... Evidence that the Bacteroides thetaiotaomicron chondroitin lyase II gene is adjacent to the chondro-4-sulfatase gene and may be ... Information on EC 3.1.6.9 - chondro-4-sulfatase. for references in articles please use BRENDA:EC3.1.6.9 ...
Deficiency3
- Maroteaux-Lamy syndrome, or mucopolysaccharidosis type VI (MPS-VI), is a lysosomal storage disorder characterized by the defective degradation of dermatan sulfate due to the deficiency of N-acetylgalactosamine-4-sulfatase (4S). (edu.au)
- OMIM 253010), based on a deficiency of different lysosomal enzymes-N-acetylgalactosamine-6-sulfate sulfatase (GALNS) and β-galactosidase, respectively. (medscape.com)
- Hunter syndrome (MPS type II) is a rare X-linked recessive disease caused by lysosomal enzyme iduronate-2-sulfatase deficiency, characterized by frequent and variable brain and skull involvement. (ajnr.org)
Mutations1
- Hunter syndrome (MPS type II, OMIM#309900 ) is a rare X-linked recessive disease caused by mutations of the gene encoding for iduronate-2-sulfatase, an enzyme involved in lysosomal GAGs catabolism. (ajnr.org)
Enzyme3
- The systematic name of this enzyme class is 4-deoxy-beta-D-gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine-4-su lfate 4-sulfohydrolase. (wikipedia.org)
- This enzyme is also called chondroitin-4-sulfatase. (wikipedia.org)
- Phosphatidylinositol-specific phospholipase C-released alkaline phosphatase was relatively stable at 40 degrees C. However, with increasing temperature from 40-60 degrees C, the enzyme was inactivated rapidly following first order kinetics and thermal inactivation constants varied from 5.08 x 10(-4) min-1 to 0.684 min-1. (heightquest.com)
Clinical1
- The severe form is characterized by clinical onset between 2 and 4 years of age, progressive neurologic involvement, and death in the first 2 decades. (ajnr.org)
Gene1
- Enzymatic and gene-based manipulations of 4-sulfation in the GAG side chains alter their ability to direct growing axons. (biologists.org)
Disease1
- Osteochondrosis, also known as osteochondritis , is related to epiphyseal dysplasia, multiple, 4 and scheuermann disease . (malacards.org)