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.
Heteropolysaccharides which contain an N-acetylated hexosamine in a characteristic repeating disaccharide unit. The repeating structure of each disaccharide involves alternate 1,4- and 1,3-linkages consisting of either N-acetylglucosamine or N-acetylgalactosamine.
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.
Proteoglycans consisting of proteins linked to one or more CHONDROITIN SULFATE-containing oligosaccharide chains.
A naturally occurring glycosaminoglycan found mostly in the skin and in connective tissue. It differs from CHONDROITIN SULFATE A (see CHONDROITIN SULFATES) by containing IDURONIC ACID in place of glucuronic acid, its epimer, at carbon atom 5. (from Merck, 12th ed)
A heteropolysaccharide that is similar in structure to HEPARIN. It accumulates in individuals with MUCOPOLYSACCHARIDOSIS.
Glycoproteins which have a very high polysaccharide content.
The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed)
Inorganic salts of sulfuric acid.
A mucopolysaccharide constituent of chondrin. (Grant & Hackh's Chemical Dictionary, 5th ed)
Enzymes which transfer sulfate groups to various acceptor molecules. They are involved in posttranslational sulfation of proteins and sulfate conjugation of exogenous chemicals and bile acids. EC 2.8.2.
A natural high-viscosity mucopolysaccharide with alternating beta (1-3) glucuronide and beta (1-4) glucosaminidic bonds. It is found in the UMBILICAL CORD, in VITREOUS BODY and in SYNOVIAL FLUID. A high urinary level is found in PROGERIA.
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.
Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate. There are two biochemically distinct forms, each due to a deficiency of a different enzyme.
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.
A non-vascular form of connective tissue composed of CHONDROCYTES embedded in a matrix that includes CHONDROITIN SULFATE and various types of FIBRILLAR COLLAGEN. There are three major types: HYALINE CARTILAGE; FIBROCARTILAGE; and ELASTIC CARTILAGE.
Oligosaccharides containing two monosaccharide units linked by a glycosidic bond.
Group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). The diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency.
A group of enzymes that catalyze the hydrolysis of various sulfate bonds of chondroitin sulfate. EC 3.1.6.-.
Large HYALURONAN-containing proteoglycans found in articular cartilage (CARTILAGE, ARTICULAR). They form into aggregates that provide tissues with the capacity to resist high compressive and tensile forces.
Enzymes which catalyze the elimination of glucuronate residues from chondroitin A,B, and C or which catalyze the hydrolysis of sulfate groups of the 2-acetamido-2-deoxy-D-galactose 6-sulfate units of chondroitin sulfate. EC 4.2.2.-.
The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.
Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.
Separation technique in which the stationary phase consists of ion exchange resins. The resins contain loosely held small ions that easily exchange places with other small ions of like charge present in solutions washed over the resins.
The lamellated connective tissue constituting the thickest layer of the cornea between the Bowman and Descemet membranes.
Acids derived from monosaccharides by the oxidation of the terminal (-CH2OH) group farthest removed from the carbonyl group to a (-COOH) group. (From Stedmans, 26th ed)
Macromolecular organic compounds that contain carbon, hydrogen, oxygen, nitrogen, and usually, sulfur. These macromolecules (proteins) form an intricate meshwork in which cells are embedded to construct tissues. Variations in the relative types of macromolecules and their organization determine the type of extracellular matrix, each adapted to the functional requirements of the tissue. The two main classes of macromolecules that form the extracellular matrix are: glycosaminoglycans, usually linked to proteins (proteoglycans), and fibrous proteins (e.g., COLLAGEN; ELASTIN; FIBRONECTINS; and LAMININ).
Carbohydrates consisting of between two (DISACCHARIDES) and ten MONOSACCHARIDES connected by either an alpha- or beta-glycosidic link. They are found throughout nature in both the free and bound form.
Bilateral hereditary disorders of the cornea, usually autosomal dominant, which may be present at birth but more frequently develop during adolescence and progress slowly throughout life. Central macular dystrophy is transmitted as an autosomal recessive defect.
A protective layer of firm, flexible cartilage over the articulating ends of bones. It provides a smooth surface for joint movement, protecting the ends of long bones from wear at points of contact.
Chromatography on non-ionic gels without regard to the mechanism of solute discrimination.
SUGARS containing an amino group. GLYCOSYLATION of other compounds with these amino sugars results in AMINOGLYCOSIDES.
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)
Unstable isotopes of sulfur that decay or disintegrate spontaneously emitting radiation. S 29-31, 35, 37, and 38 are radioactive sulfur isotopes.
The characteristic 3-dimensional shape of a carbohydrate.
An enzyme of the isomerase class that catalyzes the eliminative cleavage of polysaccharides containing 1,4-linked D-glucuronate or L-iduronate residues and 1,4-alpha-linked 2-sulfoamino-2-deoxy-6-sulfo-D-glucose residues to give oligosaccharides with terminal 4-deoxy-alpha-D-gluc-4-enuronosyl groups at their non-reducing ends. (From Enzyme Nomenclature, 1992) EC 4.2.2.7.
A group of enzymes that catalyzes the hydrolysis of terminal, non-reducing beta-D-galactose residues in beta-galactosides. Deficiency of beta-Galactosidase A1 may cause GANGLIOSIDOSIS, GM1.
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.
Electrophoresis in which cellulose acetate is the diffusion medium.
An enzyme that catalyzes the random hydrolysis of 1,4-linkages between N-acetyl-beta-D-glucosamine and D-glucuronate residues in hyaluronate. (From Enzyme Nomenclature, 1992) There has been use as ANTINEOPLASTIC AGENTS to limit NEOPLASM METASTASIS.
Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.
A group of elongate elasmobranchs. Sharks are mostly marine fish, with certain species large and voracious.
A class of inorganic or organic compounds that contain the borohydride (BH4-) anion.
A group of carbon-oxygen lyases. These enzymes catalyze the breakage of a carbon-oxygen bond in polysaccharides leading to an unsaturated product and the elimination of an alcohol. EC 4.2.2.
The sum of the weight of all the atoms in a molecule.
An enzyme that catalyzes the eliminative degradation of polysaccharides containing 1,4-beta-D-hexosaminyl and 1,3-beta-D-glucuronosyl or 1,3-alpha-L-iduronosyl linkages to disaccharides containing 4-deoxy-beta-D-gluc-4-enuronosyl groups. (Enzyme Nomenclature, 1992)
Systemic lysosomal storage disease caused by a deficiency of alpha-L-iduronidase (IDURONIDASE) and characterized by progressive physical deterioration with urinary excretion of DERMATAN SULFATE and HEPARAN SULFATE. There are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome (formerly mucopolysaccharidosis V). Symptoms may include DWARFISM; hepatosplenomegaly; thick, coarse facial features with low nasal bridge; corneal clouding; cardiac complications; and noisy breathing.
A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere.
Electrophoresis in which paper is used as the diffusion medium. This technique is confined almost entirely to separations of small molecules such as amino acids, peptides, and nucleotides, and relatively high voltages are nearly always used.
Inorganic and organic derivatives of sulfuric acid (H2SO4). The salts and esters of sulfuric acid are known as SULFATES and SULFURIC ACID ESTERS respectively.
Component of dermatan sulfate. Differs in configuration from glucuronic acid only at the C-5 position.
Compounds used extensively as acetylation, oxidation and dehydrating agents and in the modification of proteins and enzymes.
Long-chain polymer of glucose containing 17-20% sulfur. It has been used as an anticoagulant and also has been shown to inhibit the binding of HIV-1 to CD4-POSITIVE T-LYMPHOCYTES. It is commonly used as both an experimental and clinical laboratory reagent and has been investigated for use as an antiviral agent, in the treatment of hypolipidemia, and for the prevention of free radical damage, among other applications.
A proteolytic enzyme obtained from Carica papaya. It is also the name used for a purified mixture of papain and CHYMOPAPAIN that is used as a topical enzymatic debriding agent. EC 3.4.22.2.
A class of animal lectins that bind to carbohydrate in a calcium-dependent manner. They share a common carbohydrate-binding domain that is structurally distinct from other classes of lectins.
The largest class of organic compounds, including STARCH; GLYCOGEN; CELLULOSE; POLYSACCHARIDES; and simple MONOSACCHARIDES. Carbohydrates are composed of carbon, hydrogen, and oxygen in a ratio of Cn(H2O)n.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).
The N-acetyl derivative of glucosamine.
The developmental entity of a fertilized chicken egg (ZYGOTE). The developmental process begins about 24 h before the egg is laid at the BLASTODISC, a small whitish spot on the surface of the EGG YOLK. After 21 days of incubation, the embryo is fully developed before hatching.
A small leucine-rich proteoglycan that interacts with FIBRILLAR COLLAGENS and modifies the EXTRACELLULAR MATRIX structure of CONNECTIVE TISSUE. Decorin has also been shown to play additional roles in the regulation of cellular responses to GROWTH FACTORS. The protein contains a single glycosaminoglycan chain and is similar in structure to BIGLYCAN.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A beta-N-Acetylhexosaminidase that catalyzes the hydrolysis of terminal, non-reducing 2-acetamido-2-deoxy-beta-glucose residues in chitobiose and higher analogs as well as in glycoproteins. Has been used widely in structural studies on bacterial cell walls and in the study of diseases such as MUCOLIPIDOSIS and various inflammatory disorders of muscle and connective tissue.
Proteins which contain carbohydrate groups attached covalently to the polypeptide chain. The protein moiety is the predominant group with the carbohydrate making up only a small percentage of the total weight.
An analytical technique for resolution of a chemical mixture into its component compounds. Compounds are separated on an adsorbent paper (stationary phase) by their varied degree of solubility/mobility in the eluting solvent (mobile phase).
Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.
A sulfated plasma protein with a MW of approximately 66kDa that resembles ANTITHROMBIN III. The protein is an inhibitor of thrombin in plasma and is activated by dermatan sulfate or heparin. It is a member of the serpin superfamily.
Nitrous acid (HNO2). A weak acid that exists only in solution. It can form water-soluble nitrites and stable esters. (From Merck Index, 11th ed)
Techniques used to separate mixtures of substances based on differences in the relative affinities of the substances for mobile and stationary phases. A mobile phase (fluid or gas) passes through a column containing a stationary phase of porous solid or liquid coated on a solid support. Usage is both analytical for small amounts and preparative for bulk amounts.
A progressive, degenerative joint disease, the most common form of arthritis, especially in older persons. The disease is thought to result not from the aging process but from biochemical changes and biomechanical stresses affecting articular cartilage. In the foreign literature it is often called osteoarthrosis deformans.
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
The white, opaque, fibrous, outer tunic of the eyeball, covering it entirely excepting the segment covered anteriorly by the cornea. It is essentially avascular but contains apertures for vessels, lymphatics, and nerves. It receives the tendons of insertion of the extraocular muscles and at the corneoscleral junction contains the canal of Schlemm. (From Cline et al., Dictionary of Visual Science, 4th ed)
A hexose or fermentable monosaccharide and isomer of glucose from manna, the ash Fraxinus ornus and related plants. (From Grant & Hackh's Chemical Dictionary, 5th ed & Random House Unabridged Dictionary, 2d ed)
A type of ion exchange chromatography using diethylaminoethyl cellulose (DEAE-CELLULOSE) as a positively charged resin. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.
A noninflammatory, usually bilateral protrusion of the cornea, the apex being displaced downward and nasally. It occurs most commonly in females at about puberty. The cause is unknown but hereditary factors may play a role. The -conus refers to the cone shape of the corneal protrusion. (From Dorland, 27th ed)
The rate dynamics in chemical or physical systems.
Fibroblasts which occur in the CORNEAL STROMA.
The clear, viscous fluid secreted by the SYNOVIAL MEMBRANE. It contains mucin, albumin, fat, and mineral salts and serves to lubricate joints.
An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.
"Enzymes responsible for synthesis of corneal keratan sulfate glycosaminoglycans". The Journal of Biological Chemistry. 282 (41 ... "Enzymatic synthesis in vitro of the disulfated disaccharide unit of corneal keratan sulfate". The Journal of Biological ... Bartes A, Bhakta S, Hemmerich S (Apr 2001). "Sulfation of endothelial mucin by corneal keratan N-acetylglucosamine 6-O- ... "Complex-type N-linked oligosaccharides of gp120 from human immunodeficiency virus type 1 contain sulfated N-acetylglucosamine ...
"Enzymes responsible for synthesis of corneal keratan sulfate glycosaminoglycans". J. Biol. Chem. 282 (41): 30085-96. doi: ... "Entrez Gene: CHST1 carbohydrate (keratan sulfate Gal-6) sulfotransferase 1". Human CHST1 genome location and CHST1 gene details ... "Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase". J Biol Chem. 272 (51): 32321-8. doi: ... "Sulfation of sialyl N-acetyllactosamine oligosaccharides and fetuin oligosaccharides by keratan sulfate Gal-6-sulfotransferase ...
"Enzymes responsible for synthesis of corneal keratan sulfate glycosaminoglycans". J. Biol. Chem. 282 (41): 30085-96. doi: ...
Attached to each aggrecan molecule are glycosaminoglycan (GAG) chains of chondroitin sulfate and keratan sulfate. Increasing ... The amount of glycosaminoglycans (and hence water) decreases with age and degeneration. A spinal disc herniation, commonly ...
It exhibits a bottlebrush structure, in which chondroitin sulfate and keratan sulfate glycosaminoglycan (GAG) chains are ... a chondroitin sulfate and a keratan sulfate region. The three globular domains, G1, G2, and G3 are involved in aggregation, ... Barry FP, Neame PJ, Sasse J, Pearson D (1994). "Length variation in the keratan sulfate domain of mammalian aggrecan". Matrix ... This gene is a member of the lectican (chondroitin sulfate proteoglycan) family. The encoded protein is an integral part of the ...
... is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans keratan sulfate and chondroitin 6-sulfate. ... Fujimoto A, Horwitz AL (1983). "Biochemical defect of non-keratan-sulfate-excreting Morquio syndrome". Am. J. Med. Genet. 15 (2 ... Possible implication for intralysosomal catabolism of keratan sulfate". J. Biol. Chem. 271 (45): 28359-65. doi:10.1074/jbc. ... 1975). "Morquio's syndrome: deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase". Biochem. Biophys. Res. ...
It is also 6-sulfated. This sulfation is crucial to the elongation of the keratan sulfate chain. A study was done using N- ... Repeating disaccharides of glucuronic acid and galactosamine, glycosaminoglycans (CS-GAGs), are covalently coupled to the ... Like the chondroitin sulfate proteoglycans, keratan sulfate proteoglycan (KSPG) production is up regulated in reactive ... Keratan sulfate, also called keratosulfate, is formed from repeating disaccharide galactose units and N-acetylglucosamines. ...
Chondroitin sulphate/dermatan sulphate (CD/DS) and keratan sulphate (KS). In bovine corneas, the length of the CS/DS ... Proteoglycans are made of a small protein core to which one or more glycosaminoglycan (GAG) chains are attached. The GAG chains ... Macular corneal dystrophy, associated with the loss of keratan sulfate Oyster, CW (1999). "8". The human eye: structure and ...
Every person has two copies of the genes needed to break down keratan sulfate, but only one healthy copy is needed. Both ... is a rare metabolic disorder in which the body cannot process certain types of sugar molecules called glycosaminoglycans (AKA ... As such, the body is incapable breaking down keratan sulfate for disposal. The incompletely broken down GAGs remain stored in ... They both recognized the occurrence of corneal clouding, aortic valve disease, and urinary excretion of keratan sulfate. ...
... heparan sulfate, chondroitin, keratan and dermatan. Some glycosaminoglycans, such as heparan sulfate, are found attached to the ... "Selected reaction monitoring to differentiate and relatively quantitate isomers of sulfated and unsulfated core 1 O-glycans ... Factor VII Factor IX Urinary type plasminogen activator Another type of cellular glycan is the glycosaminoglycans (GAGs). These ...
... a genetic disorder Keratan sulfate, any of several sulfated glycosaminoglycans Kernel streaming, a method of processing ...
Like other glycosaminoglycans keratan sulfate is a linear polymer that consists of a repeating disaccharide unit. Keratan ... Keratan sulfate (KS), also called keratosulfate, is any of several sulfated glycosaminoglycans (structural carbohydrates) that ... Chondroitin_and_keratin_sulfate at the Duke University Health System's Orthopedics program Keratan+sulfate at the US National ... The basic repeating disaccharide unit within keratan sulfate is -3Galβ1-4GlcNAc6Sβ1-. This can be sulfated at carbon position 6 ...
Keratan sulfate I (KSI) is N -linked via a high mannose type precursor oligosaccharide. Keratan sulfate II (KSII) and keratan ... dermatan sulfate is fully hydrated whereas keratan sulfate is only partially hydrated suggesting that keratan sulfate may ... Keratan sulfates One of the main functions of the third class of GAGs, keratan sulfates, is the maintenance of tissue hydration ... transfers sulfate groups to terminal GlcNAc in keratan sulfate. The fourth class of GAG, hyaluronic acid, is not sulfated and ...
... which are proteins with side chains made of glycosaminoglycans such as keratan sulfate and chondroitin sulfate attached to ... Polysulfated glycosaminoglycan (PSGAG), sold under the brand name Adequan, is an injectable drug for dogs and horses that is ... The glycosaminoglycan side chains are polyanionic, which causes adjacent side chains to push each other away and create a " ... It is made of repeat disaccharide units (comprising hexosamine and hexuronic acid), and is similar to glycosaminoglycans ...
... of the D-galactose 6-sulfate units of the macromolecule keratan sulfate. This enzyme belongs to the family of hydrolases, ... N-acetylgalactosamine-6-sulfate sulfatase, and N-acetylgalactosamine 6-sulfatase. This enzyme participates in glycosaminoglycan ... Glossl J, Kresse H (1982). "Impaired degradation of keratan sulphate by Morquio A fibroblasts". Biochem. J. 203 (1): 335-8. PMC ... of cleaving off the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of the macromolecule chondroitin sulfate ...
Glycosaminoglycans Small proteoglycans Large proteoglycans chondroitin sulfate/dermatan sulfate decorin, 36 kDa. biglycan, 38 ... keratan sulfate fibromodulin, 42 kDa. lumican, 38 kDa Certain members are considered members of the "small leucine-rich ... heparan /chondroitin sulfate testican, 44 kDa perlecan, 400-470 kDa chondroitin sulfate bikunin, 25 kDa neurocan, 136 kDa. ... residue to which the glycosaminoglycan is joined through a tetrasaccharide bridge (e.g. chondroitin sulfate-GlcA-Gal-Gal-Xyl- ...
They attach themselves to up to 100 Chondroitin sulfate molecules and up to 50 keratan sulfate glycoaminoglycan chains. These ... King, M.W. (2014-02-10). "Glycosaminoglycans". Retrieved 2014-10-22. CS1 maint: discouraged parameter (link)[unreliable medical ... and glycosaminoglycans. They are located on the perichondrium because the perichondrium, located on the outside of developing ...
It is unique among glycosaminoglycans in that it is nonsulfated, forms in the plasma membrane instead of the Golgi apparatus, ... Sulfated, extracellular. *Chondroitin *Chondroitin sulfate. *Dermatan sulfate. *Heparan sulfate. *Keratan sulfate. Sulfated, ... without inhibiting other glycosaminoglycans) may prove useful in preventing metastasis of malignant tumor cells.[40] There is ... nonsulfated glycosaminoglycan distributed widely throughout connective, epithelial, and neural tissues. ...
Keratan sulfate[edit]. Keratan sulfates have a variable sulfate content and, unlike many other GAGs, do not contain uronic acid ... Glycosaminoglycans (GAGs) are carbohydrate polymers and mostly attached to extracellular matrix proteins to form proteoglycans ... Heparan sulfate[edit]. Heparan sulfate (HS) is a linear polysaccharide found in all animal tissues. It occurs as a proteoglycan ... Chondroitin sulfate[edit]. Chondroitin sulfates contribute to the tensile strength of cartilage, tendons, ligaments, and walls ...
Type II keratan sulphate is especially common in cartilage. Galactose or glucose sugars can be attached to a hydroxyl group of ... Proteoglycans consist of a protein with one or more sugar side chains, known as glycosaminoglycans (GAGs), attached to the ... Keratan sulphate attaches to a serine or threonine residue through GalNAc, and is extended with two galactose sugars, followed ... where the galactosphingolipid formed is often sulfated by addition of sulfate groups. One of the first and only examples of O- ...
Acharan sulfate is a heparinoid that is naturally produced by the giant African land snail, Achatina fulica. Keratan sulfate is ... Heparinoids are glycosaminoglycans which are derivatives of heparin. They include oligosaccharides and sulfated polysaccharides ... Chondroitin sulfate shows slightly less biological activity than dermatan sulfate, and is composed of O-sulfated N-acetyl-D- ... Alginic acid functions as a heparinoid when it is sulfated. Pentosan from the bark of Fagus sylvatica, when sulfated, acts with ...
chondroitin sulfate catabolic process. • keratan sulfate catabolic process. • carbohydrate metabolic process. Sources:Amigo / ... glycosaminoglycan biosynthetic process. • metabolism. • glycosphingolipid metabolic process. • ...
... the 6-sulfate groups of the N-acetyl-D-galactosamine of chondroitin sulfate and D-galactose 6-sulfate units of keratan sulfate ... Lysosomal sulfatases cleave a range of sulfated carbohydrates including sulfated glycosaminoglycans and glycolipids. Genetic ... which hydrolyzes the N-acetyl-D-glucosamine 6-sulfate units of heparan sulfate and keratan sulfate; N-sulfoglucosamine ... in the degradation of sulfated glycosaminoglycans and glycolipids in the lysosome, and in remodelling sulfated ...
... needed to break down the keratan sulfate sugar chain. Clinical features are similar in both types but appear milder in Morquio ... disorders caused by the absence or malfunctioning of lysosomal enzymes needed to break down molecules called glycosaminoglycans ... each caused by alteration of a different enzyme needed to completely break down the heparan sulfate sugar chain. Little ...
... keratan sulfate - keratinocyte growth factor - keratoacanthoma - ketoconazole - ketorolac - keyhole limpet hemocyanin - KGF - ... glycosaminoglycan - GM-CSF - GM2-KLH vaccine - GnRH - gonadotropin-releasing hormone - gonadotropin-releasing hormone agonist ... aluminium sulfate - ALVAC-CEA vaccine - Amanita phalloides - Ambien - amelanotic melanoma - Ames, Bruce - amifostine - amikacin ... chondroitin sulfate - chondrosarcoma - chordoma - chorioallantoic membrane - choriocarcinoma - choroid plexus tumor - CHPP - ...
... residues of keratan. Mediates sulfation of keratan in cornea. Keratan sulfate plays a central role in maintaining corneal ... Heparan sulfate is a glycosaminoglycan (GAG) that is linked by xylose to serine residues of proteins such as perlecan, syndecan ... Oct 1999). "A novel role for 3-O-sulfated heparan sulfate in herpes simplex virus 1 entry". Cell. 99 (1): 13-22. doi:10.1016/ ... Carbohydrate sulfotransferase D4ST1 (D4ST1), which transfers sulfate to position 4 of the GalNAc residue of dermatan sulfate. ...
... keratan sulfotransferase EC 2.8.2.22: aryl-sulfate sulfotransferase EC 2.8.2.23: (heparan sulfate)-glucosamine 3- ... glycosaminoglycan galactosyltransferase EC 2.4.1.75: deleted entry EC 2.4.1.76: deleted, included in EC 2.4.1.17 EC 2.4.1.77: ... sulfate adenylyltransferase EC 2.7.7.5: sulfate adenylyltransferase (ADP) EC 2.7.7.6: DNA-directed RNA polymerase EC 2.7.7.7: ... quercetin-3-sulfate 3'-sulfotransferase EC 2.8.2.27: quercetin-3-sulfate 4'-sulfotransferase EC 2.8.2.28: quercetin-3,3'- ...
Glycosaminoglycan biosynthesis - keratan sulfate - Reference pathway [ Pathway menu , Organism menu , Pathway entry , Hide ... Keratan sulfate (KS) is a glycosaminoglycan with the basic disaccharide unit of N-acetyllactosamine, Gal(b1-4)GlcNAc(b1-3), ... with sulfate esters at C-6 of GlcNAc and Gal residues. There are two types of KS distinguished by the protein linkage: type I ...
CS3B3, chondroitin sulphate epitope 3B3; KS5D4, keratan sulphate epitope 5D4; CS7D4, chondroitin sulphate epitope 7D4; GAG, ... Chondroitin and keratan sulphate epitopes, glycosaminoglycans, and hyaluronan in progressive versus non-progressive ... Chondroitin and keratan sulphate epitopes, glycosaminoglycans, and hyaluronan in progressive versus non-progressive ... Estimation of keratan sulphate (KS) and chondroitin sulphate (CS) epitopes may be valuable in this respect. The monoclonal ...
Keratan sulfate (KS) proteoglycan (PG) is one of the major components of the cornea, and suggested to have important roles for ... Biological function of keratan sulfate glycosaminoglycan for corneal extracellula Akama, Tomoya O. Sanford-Burnham Medical ... Biological function of keratan sulfate glycosaminoglycan for corneal extracellula. Akama, Tomoya O. / Sanford-Burnham Medical ... Biological function of keratan sulfate glycosaminoglycan for corneal extracellula. Akama, Tomoya O. / Sanford-Burnham Medical ...
Media in category "Glycosaminoglycans". The following 31 files are in this category, out of 31 total. ... Retrieved from "https://commons.wikimedia.org/w/index.php?title=Category:Glycosaminoglycans&oldid=257447280" ... Chondroitin-4-sulphate.jpg 413 × 184; 12 KB. *. Clinical case heparan sulfate analogues.jpg 960 × 496; 153 KB. ...
61, 62) The middle, or stromal, layer is largely made of collagen, keratan sulfates, and chondroitin sulfates. Keratan sulfates ... Glycosaminoglycans. Glycosaminoglycans (GAGs) are complex carbohydrates that participate in many biological processes. They can ... Lastly, chondroitin sulfate plays an important role in regeneration and plasticity in the central nervous system, meaning it is ... 28) Dermatan sulfate has been shown to aid in cell turnover and wound repair. (29) ...
"Enzymes responsible for synthesis of corneal keratan sulfate glycosaminoglycans". The Journal of Biological Chemistry. 282 (41 ... "Enzymatic synthesis in vitro of the disulfated disaccharide unit of corneal keratan sulfate". The Journal of Biological ... Bartes A, Bhakta S, Hemmerich S (Apr 2001). "Sulfation of endothelial mucin by corneal keratan N-acetylglucosamine 6-O- ... "Complex-type N-linked oligosaccharides of gp120 from human immunodeficiency virus type 1 contain sulfated N-acetylglucosamine ...
"Enzymes responsible for synthesis of corneal keratan sulfate glycosaminoglycans". J. Biol. Chem. 282 (41): 30085-96. doi: ... "Entrez Gene: CHST1 carbohydrate (keratan sulfate Gal-6) sulfotransferase 1". Human CHST1 genome location and CHST1 gene details ... "Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase". J Biol Chem. 272 (51): 32321-8. doi: ... "Sulfation of sialyl N-acetyllactosamine oligosaccharides and fetuin oligosaccharides by keratan sulfate Gal-6-sulfotransferase ...
... glycosaminoglycans such as dermatan sulfate, heparan sulfate, chondroitin sulfate and keratan sulfate; synovial fluid and ... calcium sulfate, barium sulfate, polymorphs of calcium phosphates, ceramic particles and combinations of such materials. If ... Other biological molecules, such as growth factors, glycosaminoglycans, etc., have also been identified in SIS. See: Hodde et ... heparan sulfate; DNA fragments and DNA plasmids. If other such substances have therapeutic value in the orthopaedic field, it ...
Keratan sulfate, a complex glycosaminoglycan with unique functional capability.. Caterson B, Melrose J. ... Keratan sulfate (KS)-proteoglycans and neuronal regulation in health and disease: the importance of KS-glycodynamics and ... Chondroitin sulphate and heparan sulphate sulphation motifs and their proteoglycans are involved in articular cartilage ... The heparan sulphate deficient Hspg2 exon 3 null mouse displays reduced deposition of TGF-β1 in skin compared to C57BL/6 wild ...
Glycosaminoglycan biosynthesis - keratan sulfate. *Glycosphingolipid biosynthesis - ganglio series. *Glycosphingolipid ...
Among its related pathways are Keratan sulfate/keratin metabolism and Glycosaminoglycan metabolism. Gene Ontology (GO) ...
Chondroitin and Keratan Sulfate. (Shunji Tomatsu, Tsutomu Shimada, Pravin Patel, Robert W. Mason, Tadahisa Mikami, Hiroshi ... Characterization and Chemical Modification of Glycosaminoglycans of the Extracellular Matrix. (J. Schiller, J. Becher, S. ... Agarans: Sulfated Precursors and Derivatives from Agarose, and Related Sulfated Galactans. (Marina Ciancia and María Cristina ... Heparan Sulfate and Heparin: Structure and Function. (Marcelo A. Lima, Renan P. Cavalheiro, Maria C. Z. Meneghetti, Leonardo T ...
Keratan sulfate belongs to a group of large sugar molecules called glycosaminoglycans (GAGs) or mucopolysaccharides. ... The lack of β-galactosidase activity leads to the accumulation of keratan sulfate within lysosomes. Because keratan sulfate is ... All of the mutations that cause MPS IV disrupt the breakdown of keratan sulfate by β-galactosidase. The degradation of GM1 ... GM1 ganglioside is important for normal functioning of nerve cells in the brain, and keratan sulfate is particularly abundant ...
Non-limiting examples of glycosaminoglycans include chondroitin sulfate, dermatan sulfate, keratan sulfate, hyaluronan. Non- ... Keratan sulfate. Gal or Gal(6S). GlcNAc or GlcNAc(6S). -3Gal(6S)β1-4GlcNAc(6S)β1-. Keratan sulfate type II may be fucosylated. ... an uncrosslinked keratan sulfate polymer, an uncrosslinked heparan polymer, an uncrosslinked heparan sulfate polymer, or an ... however heparan sulfates disaccharide units are organized into distinct sulfated and non-sulfated domains.. ...
This enzyme is indispensable for the degradation of the glycosaminoglycans (GAGs) keratan sulfate and chondroitin-6-sulfate. ... Recombinant human N-acetylgalactosamine-6-sulfate sulfatase (GALNS) produced in the methylotrophic yeast Pichia pastoris. Sci ... Characterization of a recombinant N-acetylgalactosamine-6-sulfate sulfatase produced in E. coli for enzyme replacement therapy ... Effect of culture conditions and signal peptide on production of human recombinant N-acetylgalactosamine-6-sulfate sulfatase in ...
The body needs these enzymes to break down long strands of sugar molecules called keratan sulfate. In both types, abnormally ... These chains of molecules are called glycosaminoglycans (formerly called mucopolysaccharides). As a result, the molecules build ... large amounts of glycosaminoglycans build up in the body. This can damage organs. ...
Mucopolysaccharides including Chondroitin sulfate, Dermatan sulfate, Heparin, Heparan sulfate, Hyaluronic acid, Keratan sulfate ... Glycosaminoglycans ( ... Sulfur containing (e.g., sulfides, sulfones, sulfates, ...
BP] glycosaminoglycan metabolic process *[BP] keratan sulfate biosynthetic process *[BP] keratan sulfate metabolic process *[BP ...
Glycosaminoglycans (GAGs). Hyaluronic acid (the primary GAG in loose CT). small, highly expanded. hydrophilic and negatively ... Proteoglycans (heparin sulphate). -Collagen IV (+ other collagens). -Laminin and other minor proteins ... Heparan-, keratan-sulfate) linked to proteins 27 what are the steps of wound healing? ...
The binding of Langerin to glycosaminoglycans, especially heparin, has been extensively studied and the high-affinity binding ... Keratan sulfate in emphysema and COPD.. (A) The structure of keratan sulfate, KS-1, L4 consists of galactose-6-sulfate unit and ... Keratan sulfate in emphysema and COPD.. (A) The structure of keratan sulfate, KS-1, L4 consists of galactose-6-sulfate unit and ... Identification of keratan sulfate disaccharide at C-3 position of glucuronate of chondroitin sulfate from Mactra chinensis ...
This protein contains both chondroitin sulfate and keratan sulfate glycosaminoglycans. It functions as a boundary lubricant at ...
a. Dermatan sulfate. b. Proteoglycan. c. Glycosaminoglycan. d. Chondroitin sulfate. e. Keratan sulfate ...
Chapter 2 - Chondroitin and Keratan Sulfate. (Shunji Tomatsu, Tsutomu Shimada, Pravin Patel, Robert W. Mason, Tadahisa Mikami, ... Chapter 3 - Characterization and Chemical Modification of Glycosaminoglycans of the Extracellular Matrix. (Schiller J, Becher J ... Chapter 9 - Agarans: Sulfated Precursors and Derivatives from Agarose, and Related Sulfated Galactans. (Marina Ciancia and ... Sulfated Polysaccharides. Miguel Gama. Minho University, Biological Engineering Department, Braga, Portugal. Series: ...
Glycosaminoglycan biosynthesis - keratan sulfate. *Glycosphingolipid biosynthesis - lacto and neolacto series. *Lactose ...
Glycosaminoglycan metabolism (Homo sapiens) * Keratan sulfate/keratin metabolism (Homo sapiens) * Keratan sulfate biosynthesis ... B3GNT1,2,3,4,7 add GlcNAc to form Keratan-PG (Homo sapiens) * B3GNT1,2,3,4,7 [Golgi membrane] (Homo sapiens) * B3GNT7 [Golgi ...
Glycosaminoglycan metabolism (Homo sapiens) * Keratan sulfate/keratin metabolism (Homo sapiens) * Keratan sulfate biosynthesis ... CHST2,5,6 transfer SO4(2-) to GlcNAc residues on keratan-PG to form KSPG (Homo sapiens) * KSPG(1) [Golgi lumen] (Homo sapiens) ...
keratan sulfate 30 July 2008 See also glycosaminoglycans (GAGs). * glucose 2 June 2004 Definition: The glucose (Glc) is a ... chondroitin sulfate 16 October 2006 Definition: Chondroitin sulfate is a sulfated glycosaminoglycan (GAG) composed of a chain ... Chondroitin sulfate is the most prevalent glycosaminoglycan (mucopolysaccharide). A chondroitin chain can have over 100 ... Chondroitin sulfate is an important structural component of cartilage and provides much of its resistance to compression. (...) ...
two major glycosaminoglycans w/ in cartilage ground substance consist of chondroitin sulfate and keratan sulfate;. - both of ... keratan sulfate:. - disaccharide repeating unit consisting of N-acetyl glucosamine alternating w/ galactose;. - keratan sulfate ... there are about 100 chondroitin sulfate glycosaminoglycan chains per proteoglycan subunit;. - chondroitin sulfate is the most ... Synthesis of chondrocytic keratan sulphate-containing proteoglycans by human chondrosarcoma cells in long-term cell culture.. ...
Their protein backbones are linked to different glycosaminoglycans, such as dermatan-, chondroitin-, keratan- or heparan ... Objectives: The expression patterns of various cell-associated heparan and chondroitin/dermatan-sulphate PGs in human skin and ... sulphate. The molecules have specific functions during developmental processes as well as in diseases, such as cancer and ...
Discontinuous nature of the protein-keratan sulphate core of cartilage proteoglycan. *. Concentration, distribution, and ... Effect of in vitro incubation on distribution and synthesis of glycosaminoglycans of articular cartilage ... Overlabelling of chondroitin sulphate (ChS) chains after application of specific inhibitors of their biosynthesis ... Passive agglutination method using collagen-coated tanned sheep erythrocytes to demonstrate collagen-glycosaminoglycan ...
  • KS-PG is consists of two different components, core proteins and attached carbohydrates called glycosaminoglycans (GAGs). (grantome.com)
  • Keratan sulfate belongs to a group of large sugar molecules called glycosaminoglycans (GAGs) or mucopolysaccharides. (medlineplus.gov)
  • This enzyme is indispensable for the degradation of the glycosaminoglycans (GAGs) keratan sulfate and chondroitin-6-sulfate. (nature.com)
  • Glycosaminoglycans [1] ( GAGs ) or mucopolysaccharides [2] are long unbranched polysaccharides consisting of a repeating disaccharide unit. (wikipedia.org)
  • Keratan sulfate (KS) is one of the glycosaminoglycans (GAGs), a family of polysaccharides consisting of repeats of disaccharide units. (go.jp)
  • Glycosaminoglycans (GAGs) are distributed in the whole body and play a variety of important physiological roles associated with inflammation, growth, coagulation, fibrinolysis, lipolysis, and cell-matrix biology. (omicsonline.org)
  • GAGs (except for hyaluronan) are sulfated polysaccharides made of repeating disaccharides, consisting of uronic acid (or galactose) and hexosamines. (omicsonline.org)
  • The proteoglycan molecule is made of a central protein core with side chains of glycosaminoglycans (GAGS. (animalwellnessmagazine.com)
  • This study examined systematic antibodies reactive to glycosaminoglycans (GAGs), the carbohydrate components of proteoglycans that are released in large amounts from degrading cartilage. (biomedcentral.com)
  • The present study focuses on NAbs that are reactive to glycosaminoglycans (GAGs), important molecular constituents of both cell surface proteoglycans and large and small proteoglycans of the extracellular matrix of hyaline cartilage. (biomedcentral.com)
  • GALNS deficiency induces the accumulation of glycosaminoglycans (GAGs), keratan sulfate (KS), and chondroitin-6-sulfate (C6S) in multiple tissues, particularly bone, cartilage, heart valves, and cornea, while β-galactosidase deficiency induces the accumulation of only KS in those tissues. (medscape.com)
  • These GAGs include keratan sulfate (made from glucosamine), and chondroitin sulfate, both being very common oral joint supplements. (marvistavet.com)
  • There are five different types of GAGs: chondroitin 4-sulphate, chondroitin 6-sulphate, heparan sulphate, dermatan sulphate, and keratan sulphate. (gopetsamerica.com)
  • The most common types of GAGs in cartilage are chondroitin sulfate and keratan sulfate, both of which are found in aggrecan. (newworldencyclopedia.org)
  • Glycosaminoglycans (GAGs) are distinct from other sugars/oligosaccharides in that they are polymers of disaccharide units composed of an amino sugar, N-acetylglucosamine (GlcNAc), or N-acetylgalactosamine (GalNAc), and uronic acid, glucuronic acid (GlcUA), or iduronic acid (IdUA). (unl.edu)
  • There are four classes of GAGs (Figure 1): Chondroitin (Chon) sulfate (CS) A through E, heparin (HP) and heparan sulfate (HS), KS, and hyaluronan (HA). (unl.edu)
  • All of the GAGs, except HA and some KS chains (as noted above), are attached to and assembled as O-linked glycans on proteoglycan core proteins and are sulfated to various degrees. (unl.edu)
  • The mucopolysaccharidoses (MPSs) are a group of 11 distinct metabolic disorders that result from the deficiency of the various lysosomal enzymes required to degrade the glycosaminoglycans (GAGs) that constitute a major component of the extracellular matrix, joint fluid and connective tissue. (els.net)
  • To monitor glycosaminoglycans (GAGs) in patients previously diagnosed with MPS, refer to Mucopolysaccharides, Quantitative, Urine ( 0081357 ). (aruplab.com)
  • Keratan sulfate (KS) proteoglycan (PG) is one of the major components of the cornea, and suggested to have important roles for corneal development and maintenance of transparency of the tissue. (grantome.com)
  • Keratan sulfate may modify core proteins through N-linked glycosylation or O-linked glycosylation of the proteoglycan. (wikipedia.org)
  • 2015. The transmembrane heparan sulphate proteoglycan syndecan-4 is involved in establishment of the lamellar structure of the annulus fibrosus of the intervertebral disc . (cardiff.ac.uk)
  • There are several different proteoglycan molecules in the joint cartilage including chondroitin sulfate and keratan sulfate. (animalwellnessmagazine.com)
  • Lumican is the major keratan sulfate proteoglycan of the cornea but is also distributed in interstitial collagenous matrices throughout the body. (bio-rad.com)
  • The term proteoglycan is given to the macromolecule composed of a central protein core to which are attached glycosaminoglycan chains. (docme.ru)
  • Addition to the culture medium of a chondroitin 6-sulphate/keratan sulphate containing PG (BNC-PG) markedly enhanced the cathodal re-orientation of embryonic Xenopus neurites in an electric field, whereas a proteoglycan containing chondroitin 4-sulphate (RC-PG) was inhibitory. (biologists.org)
  • The presence of the glycosaminoglycan side chains of heparan sulfate proteoglycan, an important constituent of the glomerular basement membrane, is decreased in DKD proportionally to the increasing degree of proteinuria. (cochrane.org)
  • A gene on chromosome 10q22.1 that encodes a sulfotransferase which transfers sulfate to chondroitin, yielding chondroitin sulfate, a predominant proteoglycan of cartilage, cell surfaces and extracellular matrices. (thefreedictionary.com)
  • [5] Heparin / heparan sulfate (HSGAGs) and chondroitin sulfate / dermatan sulfate (CSGAGs) are synthesized in the Golgi apparatus , where protein cores made in the rough endoplasmic reticulum are posttranslationally modified with O-linked glycosylations by glycosyltransferases forming proteoglycans . (wikipedia.org)
  • Heparin is a mucopolysaccharide composed of amino sugar and uronic acid residues which is obtained from beef, porcine, sheep, whale, or other mammalian tissue by extraction with a solution of potassium acetate, alkaline ammonium sulfate and the like. (google.com)
  • The complex carbohydrate macromolecules named glycosaminoglycans are represented by heparin, heparan sulfate, dermatan sulfate, chondroitin sulfates, hyaluronic acid and keratan sulfate and by their derivatives. (unipv.it)
  • Heparin and related substances, including glycosaminoglycans, chondroitin, low-molecular-weight heparin (LMWH), heparinoids, heparitin sulfate, hyaluronic acid and keratan sulphate, have been demonstrated a few positive effects on delaying the progression of diabetic kidney disease (DKD). (cochrane.org)
  • These chains of molecules are called glycosaminoglycans (formerly called mucopolysaccharides). (medlineplus.gov)
  • MPS is defined by the body's inability to break down large sugar chains called glycosaminoglycans (formerly known as mucopolysaccharides). (everydayhealth.com)
  • Differential Staining of Acid Glycosaminoglycans (Mucopolysaccharides) by Alcian Blue in Salt Solutions. (patentgenius.com)
  • Mucopolysaccharides are complex sulphated sugars with repeating disaccharides that form sulphated glycosaminoglycans (GAG). (gopetsamerica.com)
  • The degradation of glycosaminoglycans (or mucopolysaccharides) takes place in lysosomes that contain the acid hydrolases. (els.net)
  • Mucopolysaccharides (Glycosaminoglycans) include: Keratan Sulfate, Heparan Sulfate, Dermatan Sulfate, and Chondroitin Sulfates 4 and 6. (aruplab.com)
  • Zhang H, Muramatsu T, Murase A, Yuasa S, Uchimura K, Kadomatsu K (2006) N-Acetylglucosamine 6-O-sulfotransferase-1 is required for brain keratan sulfate biosynthesis and glial scar formation after brain injury. (springer.com)
  • Seko A., Yamashita K. (2008) Biosynthesis of Keratan Sulfate. (springer.com)
  • We selected most pathways B4GALT4 participated on our site, such as Glycosaminoglycan biosynthesis - keratan sulfate, Glycosphingolipid biosynthesis - lacto and neolacto series, Metabolic pathways, which may be useful for your reference. (creativebiomart.net)
  • Arising from numerous modifications during biosynthesis, heparan sulfate is characterized by extensive structural variability that affects interactions with various proteins and thus influences important biological functions. (diva-portal.org)
  • GM1 ganglioside is important for normal functioning of nerve cells in the brain, and keratan sulfate is particularly abundant in cartilage and the clear covering of the eye (cornea). (medlineplus.gov)
  • Because keratan sulfate is predominantly found in cartilage and the cornea, the buildup of this substance causes skeletal abnormalities and cloudy corneas. (medlineplus.gov)
  • Chondroitin sulfate is an important structural component of cartilage and provides much of its resistance to compression. (humpath.com)
  • The matrix (ground substance) of cartilage is composed of proteoglycans and glycosaminoglycans. (histology-world.com)
  • Chondroitin sulfate is the major glycosaminoglycan found in cartilage. (animalwellnessmagazine.com)
  • Chondroitin sulfate and keratan sulfate in guinea pig costal cartilage, nasal septum cartilage and nucleus pulposus were separated and fractionated by chromatography on CPC-cellulose, ECTEOLA-cellulose and Sephadex G-200 columns. (lu.se)
  • Serum keratan sulfate in rats was detected by HPLC and transiently increased (peaked at 3 to 4 weeks) along with depletion of keratan sulfate in cartilage tissue. (biomedcentral.com)
  • As part of aggrecan, chondroitin sulfate is a major component of cartilage . (bionity.com)
  • The tightly packed and highly charged sulfate groups of chondroitin sulfate generate electrostatic repulsion that provides much of the resistance of cartilage to compression. (bionity.com)
  • It is the high glycosaminoglycan content of the matrix which confers these properties on cartilage, as will be discussed later on. (docme.ru)
  • In cartilage the latter consist of about 80 per cent of chondroitin SUlphate and 20 per cent of keratan SUlphate [3]. (docme.ru)
  • Both these molecules contain acidic groups (carboxyl and/or SUlphate) which confer on cartilage a net negative fixed charge. (docme.ru)
  • The water content of cartilage lies in the range 65-80% of the wet weight of tissue, collagen in the range 20-25% and glycosaminoglycans in the range 1· 5-6% [3-7J. (docme.ru)
  • Deficiency of this enzyme progressively causes accumulation of the glycosaminoglycans keratan sulfate and chondroitine-6-sulfate in multiple tissues (especially bone, cartilage, heart valves, and cornea), and leads to a unique form of skeletal dysplasia. (mpssociety.org)
  • The expression patterns of various cell-associated heparan and chondroitin/dermatan-sulphate PGs in human skin and chronic venous ulcers were investigated. (nih.gov)
  • Keratan sulphate, a minor component of BNC-PG, was found to be inhibitory, whereas dermatan sulphate, an epimer of chondroitin 4-sulphate, had no effect. (biologists.org)
  • Expressions of heparan sulphate, chondroitin sulphate, dermatan sulphate and their fragments were analyzed by electrospray ionization mass spectrometry, with the technique for extraction and quantification of glycosaminoglycans after proteolysis and electrophoresis. (scielo.br)
  • Neoplastic tissues showed greater amounts of chondroitin sulphate and dermatan sulphate compared to non-neoplastic tissues, while heparan sulphate was decreased in neoplastic tissues. (scielo.br)
  • Quantitative biosynthetic studies with cultures highly enriched for glial fibrillary acidic protein (GFAP+) cells of neonatal mammalian brain demonstrated production of four proteoglycans: hyaluronate (HA), heparan sulphate (HS), chondroitin sulphate (CS), and dermatan sulphate (DS). (semanticscholar.org)
  • In the human body, there are several types of protein modifications that involve glycans being attached to glycoproteins, such as N -glycosylation, O -glycosylation, glycosylphosphatidylinositol (GPI)-anchor and glycosaminoglycan chains and others attaching to protein molecules [ 1 ]. (portlandpress.com)
  • Within lysosomes, β-galactosidase helps break down certain molecules, including substances called GM1 ganglioside and keratan sulfate. (medlineplus.gov)
  • The body needs these enzymes to break down long strands of sugar molecules called keratan sulfate. (medlineplus.gov)
  • Chondroitin sulfate proteoglycans (CSPGs) are large extracellular matrix molecules which are highly upregulated in the glial scar after injury to the nervous system. (go.jp)
  • 3. The method of claim 1 wherein said prosthetic meniscus fabricated in step (a) comprises a plurality of glycosaminoglycan molecules. (google.com)
  • In these bifunctional molecules the protein moiety binds collagen fibrils and the highly charged hydrophilic glycosaminoglycans regulate interfibrillar spacings. (bio-rad.com)
  • The core proteins (shown in green) have bottlebrush like GAG (glycosaminoglycan) molecules coming off them ready to soak up water. (marvistavet.com)
  • The matrix is mainly composed of proteoglycans, which are large molecules with a protein backbone and glycosaminoglycan (GAG) side chains. (newworldencyclopedia.org)
  • This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate. (novusbio.com)
  • OBJECTIVE To determine if a single time point estimation of chondroitin sulphate (CS) or keratan sulphate (KS) epitopes, hyaluronan (HA), or total glycosaminoglycans (GAG) in knee synovial fluid at time of hospital referral can predict subsequent radiographic progression of knee osteoarthritis. (bmj.com)
  • Hyaluronic acid (HA), also known as hyaluronan, is a non-sulfated glycosaminoglycan that is distributed widely throughout the human body in connective, epithelial, and neural tissues. (google.com.au)
  • We investigated whether osteoarthritic change could be obtained in a rat strenuous running model, whether serum keratan sulfate in rats could be detected by HPLC and was associated with onset or progression of osteoarthritis, and whether hyaluronan injection suppressed development of osteoarthritis and elevation of serum keratan sulfate. (biomedcentral.com)
  • Hyaluronan treatment suppressed morphological progression of osteoarthritis and elevation of serum keratan sulfate. (biomedcentral.com)
  • Weekly intraarticular injection of hyaluronan controlled the development of osteoarthritis, and the effect was reflected by serum keratan sulfate. (biomedcentral.com)
  • Hyaluronan is also a glycosaminoglycan. (biomedcentral.com)
  • Keratan sulfate (KS) is a glycosaminoglycan with the basic disaccharide unit of N-acetyllactosamine, Gal(b1-4)GlcNAc(b1-3), with sulfate esters at C-6 of GlcNAc and Gal residues. (genome.jp)
  • Glycosaminoglycans are long unbranched polysaccharides consisting of a repeating disaccharide unit (saccharide is a synonym for sugars ). (newworldencyclopedia.org)
  • Glycosaminoglycans have high degrees of heterogeneity with regards to molecular mass, disaccharide construction, and sulfation due to the fact that GAG synthesis, unlike proteins or nucleic acids, is not template driven, and dynamically modulated by processing enzymes. (wikipedia.org)
  • KS covalently attaches to scaffold core proteins to yield keratan sulfate proteoglycans (KSPGs). (go.jp)
  • 11. Proteoglycans are complexes of proteins and glycosaminoglycans that form feathery structures. (oregonstate.edu)
  • Chondroitin sulfate chains are linked to hydroxyl groups on serine residues of certain proteins. (bionity.com)
  • Exactly how proteins are selected for attachment of glycosaminoglycans is not understood. (bionity.com)
  • All of the glycosaminoglycans were associated with core proteins that were integral to the cell membrane and associated with the cell surface by non-covalent interactions involving glycosaminoglycans. (semanticscholar.org)
  • 2010. Chondroitin sulphate sulfation motifs in intervertebral disc development [Abstract] . (cardiff.ac.uk)
  • 10. Glycosaminoglycans are polysaccharides that contain either N-acetylgalactosamine or N-acetylglucosamine as one of their monomeric units. (oregonstate.edu)
  • Polysaccharides (glycos-aminoglycans), especially heparan sulfate, are significant components of all types of amyloid deposits. (diva-portal.org)
  • Kitayama K, Hayashida Y, Nishida K, Akama TO (2007) Enzymes responsible for synthesis of corneal keratan sulfate glycosaminoglycans. (springer.com)
  • The analysis revealed that E5 could modify glycan sialylation, the N‑glycosylation pathway, keratan sulfate and glycosaminoglycan synthesis. (spandidos-publications.com)
  • May take part in heparan sulfate synthesis by supplying UDP-Glc-NAc, the donor substrate, and thus be involved in growth factor signaling. (mybiosource.com)
  • OMIM 253010), based on a deficiency of different lysosomal enzymes-N-acetylgalactosamine-6-sulfate sulfatase (GALNS) and β-galactosidase, respectively. (medscape.com)
  • Mucopolysaccharidosis IVA (MPS IVA, also known as Morquio A Syndrome) is a disease characterized by deficient activity of N-acetylgalactosamine-6-sulfatase (GALNS) causing excessive lysosomal storage of glycosaminoglycans such as keratan sulfate and chondroitin sulfate. (cnbc.com)
  • 17 Galactosamine (N-Acetyl)-6-Sulfate Sulfatase (GALNS) ELISA Kits from 5 manufacturers are available on www.antibodies-online.com. (antibodies-online.com)
  • Mucopolysaccharidosis IVA (MPS IVA: Morquio A syndrome) is caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase. (mpssociety.org)
  • Heparan sulfate proteoglycans (HSPGs) are constituents of the extracellular matrix, and through their heparan sulfate chains and protein core, modulate multiple events that occur during the metastatic cascade. (frontiersin.org)
  • These effects of BNC-PG and RC-PG were reproduced by their chondroitin sulphate glycosaminoglycan side chains alone. (biologists.org)
  • We conclude that in vitro specific interactions between these two nerve guidance cues do occur and that the specificity of the response is critically dependent on the charge pattern of the proteoglycans chondroitin sulphate side chains. (biologists.org)
  • The expression of a host of proteoglycans with differing glycosaminoglycan side chains varies in both time and place in the developing nervous system, thus the scope is vast for spatial and temporal modulation of nerve guidance by interacting cues. (biologists.org)
  • Stepwise degradation of the main glycosaminoglycan chains, heparan sulfate (HS), dermatan sulfate (DS) and keratan sulfate (KS), as they are degraded by the indicated enzymes (the degradation pathway of chondroitin sulfates, being similar to that of DS is not shown). (els.net)
  • Distinct chemical differences were found between the total fractions of chondroitin sulfate from the three tissues. (lu.se)
  • In addition, the existence of a second metabolic component with a faster turnover (half-life about 3 days) was indicated in chondroitin sulfate from all three tissues. (lu.se)
  • Within the total fractions of both chondroitin sulfate and keratan sulfate from the three tissues studied, a considerable degree of metabolic heterogeneity was apparent between, and within, subfractions of differing molecular weight. (lu.se)
  • To determine the presence of glycosaminoglycans in the extracellular matrix of connective tissue from neoplastic and non-neoplastic colorectal tissues, since it has a central role in tumor development and progression. (scielo.br)
  • Electrospray ionization mass spectrometry showed characteristic disaccharide fragments from glycosaminoglycans, indicating their structural characterization in the tissues analyzed. (scielo.br)
  • The basement membrane-specific heparan sulfate proteoglycans perlecan ( 9 ) and agrin ( 10 ) are components of the GBM. (asnjournals.org)
  • The deficiency of any one of 11 acid hydrolases, required to normally degrade the glycosaminoglycans, gives rise to the group of lysosomal storage disorders known as the mucopolysaccharidoses. (els.net)
  • Mucopolysaccharidoses are a group of metabolic disorders in which abnormal accumulations of glycosaminoglycans occur because of enzyme deficiencies. (wikipedia.org)
  • While elongating, the HSGAG is dynamically modified, first by N-deacetylase, N-sulfotransferase ( NDST1 ), which is a bifunctional enzyme that cleaves the N-acetyl group from GlcNAc and subsequently sulfates the N-position. (wikipedia.org)
  • In 1974, GALNS enzyme and its deficiency were discovered and identified using oligosaccharide substrate prepared from C6S containing N-acetylgalactosamine-6-sulfate. (medscape.com)
  • Glycosaminoglycan (GAG), chondroitin sulfate (CS), keratan sulfate (KS), and hyaluronic acid (HA) contents were quantitatively analyzed by enzyme-linked immunosorbent assay. (springer.com)
  • Inhibition-enzyme-linked immunosorbent assay also was used to detect keratan sulfate in the aqueous humor. (lancs.ac.uk)
  • Fukuta M, Inazawa J, Torii T, Tsuzuki K, Shimada E, Habuchi O (1997) Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase. (springer.com)
  • Characterization of the chondroitin sulfates included determination of molecular weight and of the number, and position, of sulfate groups of the disaccharide units. (lu.se)
  • 5. Characterization of glycosaminoglycans in corneal stem cells in MCD subjects. (unipv.it)
  • 8. Fine characterization of glycosaminoglycans in a animal model of Alzheimer's disease. (unipv.it)
  • 9. Fine characterization of glycosaminoglycans in human mitral and aortic valves. (unipv.it)
  • The Department in which Prof. Nicola Volpi and Dr. Francesca Maccari perform their studies and researches is equipped with HPLC with UV and fluorimetric detectors and online ESI-MS for separation and characterization of macromolecules in size-exclusion chromatography, and of glycosaminoglycan disaccharides by strong anion-exchange chromatography. (unipv.it)
  • Characterization of glycosaminoglycans produced by primary astrocytes in vitro. (semanticscholar.org)
  • Without either of these enzymes, the body cannot break down certain glycosaminoglycans, particularly keratan sulfate. (everydayhealth.com)
  • These enzymes are involved in the degradation of specific glycosaminoglycans. (ggc.org)
  • CHST3 can also transfer sulfate to keratan, another glycosaminoglycan, and to sialyl N-acetyllactosamine oligosaccharides. (thefreedictionary.com)
  • the latter was contaminated with protein and possibly a small amount of another glycosaminoglycan. (portlandpress.com)
  • On the other hand keratan sulfate, another glycosaminoglycan, identified biochemically and by immunohistochemical analysis in Alzheimer diseased cerebral cortex, was reduced to less than half of the amount in control tissue. (diva-portal.org)
  • The lack of β-galactosidase activity leads to the accumulation of keratan sulfate within lysosomes. (medlineplus.gov)
  • The progressive accumulation of glycosaminoglycans, a major component of the extracellular matrix, joint fluid and connective tissue, leads ultimately to multisystem involvement including organomegaly, dysostosis multiplex and abnormal facies. (els.net)
  • Among its related pathways are Keratan sulfate/keratin metabolism and Glycosaminoglycan metabolism . (genecards.org)
  • Next, C-5 uronyl epimerase coverts d-GlcA to l-IdoA followed by 2- O sulfation of the uronic acid sugar by 2- O sulfotransferase ( Heparan sulfate 2-O-sulfotransferase ). (wikipedia.org)
  • The exception is keratan sulfate (KS) in which the uronic acid is replaced by the neutral sugar galactose. (unl.edu)
  • Glycosaminoglycans in urine from patients representing the major different mucopolysaccharidoses were separated and measured by use of a procedure that requires only 2 mL of urine. (elsevier.com)
  • Moser, H. W. / Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses . (elsevier.com)
  • KS-GAG is a linearly elongated carbohydrate chain that consists of repeating disaccharide units of 3Gal~1-4GlcNAc~1- (Gal, galactose;GlcNAc, Nacetylglucosamine), with sulfate on 6-0 position of Gal and GlcNAc. (grantome.com)
  • The repeating unit (except for keratan ) consists of an amino sugar ( N -acetylglucosamine or N -acetylgalactosamine ) along with a uronic sugar ( glucuronic acid or iduronic acid ) or galactose . (wikipedia.org)
  • Glucosamine hydrochloride and glucosamine sulfate are more effective than N-acetylglucosamine. (animalwellnessmagazine.com)
  • Along with glucosamine , chondroitin sulfate has become a widely used dietary supplement for treatment of osteoarthritis . (bionity.com)
  • Note that although GM2 gangliosidosis (depicted in grey) results from a deficiency of β‐hexosaminidase, also required for glycosaminoglycan degradation, it is not considered an MPS sensu stricto since its clinical symptoms are due principally to defective sphingolipid degradation. (els.net)
  • Their protein backbones are linked to different glycosaminoglycans, such as dermatan-, chondroitin-, keratan- or heparan sulphate. (nih.gov)
  • What are the different glycosaminoglycans that exist in the ECM? (brainscape.com)
  • This test includes quantitative measurement of total glycosaminoglycans as well as quantitation of the individual GAG components, including heparan sulfate, dermatan sulfate, chondroitin sulfate, and keratan sulfate. (ggc.org)
  • Electrophoretic chambers for SDS-PAGE electrophoresis, chambers for agarose-gel electrophoresis for the separation of various molecular mass proteoglycans and glycosaminoglycans. (unipv.it)
  • Chondroitin , without the "sulfate", has been used to describe a fraction with little or no sulfation. (bionity.com)
  • have suggested that all such preparations of chondroitin sulfate be referred to as "sodium chondroitin" regardless of their sulfation status. (bionity.com)
  • HP is typically more highly sulfated than HS and is primarily found in mast cells of the immune system. (unl.edu)
  • In contrast, HS is ubiquitous throughout the body and its sugars are highly sulfated in patches along the chain, allowing differing ligands to bind to regions of higher or lower anionic charge density. (unl.edu)
  • Light microscopy of the corneas revealed deposits of glycosaminoglycans in keratocytes, endothelial cells and extracellularly within the stroma, all staining positively with Alcian blue and periodic acid-Schiff. (harvard.edu)
  • Changes in the distribution of keratan sulfate and chondroitin sulfate on endothelial cell surfaces were investigated on organ cultured bovine corneas during endothelial wound healing. (lancs.ac.uk)
  • Changes in the levels of keratan sulfate during endothelial wound healing were investigated in organ cultured human corneas and in vivo in rabbit corneas. (lancs.ac.uk)
  • Wound healing experiments resulted in changes of keratan sulfate levels on the migrating endothelial cells in bovine, human, and rabbit. (lancs.ac.uk)
  • In wounded organ cultured bovine corneas, there was a decrease in keratan sulfate levels and an increase in chondroitin sulfate levels on migrating endothelial cells. (lancs.ac.uk)
  • The pattern of occurrence of keratan sulfate and chondroitin sulfate on the corneal endothelial cells in normal and wounded cornea suggests that these glycosaminoglycans have differing roles in endothelial adhesion and migration. (lancs.ac.uk)
  • Serum keratan sulfate was associated with progression of osteoarthritis. (biomedcentral.com)
  • The second study purpose was to investigate serum keratan sulfate in rats by HPLC and its association with onset or progression of osteoarthritis. (biomedcentral.com)
  • Although the name "chondroitin sulfate" suggests a salt with a sulfate counter-anion, this is not the case, as sulfate is covalently attached to the sugar. (bionity.com)
  • If you have signs or symptoms of MPS, your doctor will likely order a urine test to see if you have high levels of glycosaminoglycans. (everydayhealth.com)
  • Glycosaminoglycans are typically elevated in the urine of affected patents. (ggc.org)
  • For patients with a suspected MPS diagnosis, measurement of glycosaminoglycans in urine can be a useful screening test. (ggc.org)
  • Proteoglycans are composed of a protein core and attached glycosaminoglycans. (histology-world.com)
  • Improved Quantitation and Discrimination of Sulphated Glycosaminoglycans by Use of Dimethylmethylene Blue. (patentgenius.com)
  • Definition: Chondroitin sulfate is a sulfated glycosaminoglycan (GAG) composed of a chain of alternating sugars (N-acetylgalactosamine and glucuronic acid). (humpath.com)
  • Capillary electroforesis for the determination of glycosaminoglycan unsaturated disaccharides. (unipv.it)
  • Homeostasis of extracellular matrix depends on the integrity and assembly of collagens, glycoproteins, proteoglycans (PG), and sulfated glycosaminoglycans (S-GAG). (scielo.br)