Keratan sulfate skeletal

Two types of keratan sulfate, corneal and skeletal, have been differentiated by the hydrolytic behavior of the linkage region. The former has an N-... 

Nieduszynski lA, Huckerby TN, Dickenson JM, Brown GM, Tai GH, Morris HG, Eady S. There are 2 major types of skeletal keratan sulfates. Biochem. J. 1990 271(l) 243-245. 

Skeletal keratan sulfate and comeal keratan sulfate are linked to core protein through 0-linked and A-linked oligosaccharides, respectively, of the general types described in O Sect. 2.4 and O Sect. 2.5. 

MPS IV A Morquio A Distinctive skeletal abnormalities, corneal clouding, odontoid hypoplasia milder forms known to exist Galactose 6-sulfatase Keratan sulfate, chondroitin 6-sulfate... 

Skeletal keratan sulfate and corneal keratan sulfate chains are attached to core protein through O-linked oligosaccharides [29] and N-linked oligosaccharides [30,31] respectively, identical to O-linked and N-linked oligosaccharides of the general class of glycoproteins. 

Plaas AH, West LA, Thonar EJ, Karcioglu ZA, Smith CJ, Klintworth GK, Hascall VC. Altered fine structures of corneal and skeletal keratan sulfate and chondroitin/dermatan sulfate in macular corneal dystrophy. J Biol Chem 2001 276 39788-39796. 

In all, eight glycosaminoglycans of essentially diflFerent chemical structures have been identified. Through the times, these glycosaminoglycans have been individually named in a number of ways, as shown in Table 1. Most of these names are used currently, and so the reader will find it useful to have this table available when consulting the primary literature. Where the term chondroitin sulfate appears in the more recent literature, this can mean chondroitin 4-sulfate or chondroitin 6-sulfate or a mixture of the two. The terms keratan sulfate I and keratan sulfate II are sometimes used to denote keratan sulfates of comeal and skeletal origin, respectively, there being some differences between the two. 

There are two forms of Morquio syndrome. The Morquio syndrome, type A is characterized by significant physical and skeletal abnormalities after birth with no neurological problems in childhood. Corneal clouding, enlarged liver, development of abnormal teeth, and hearing loss occur later in the course of the disease. Urinary excretion of increased quantities of keratan sulfate and/ or chondroitin-6-sulfate is strongly suggestive of the Morquio syndrome. The prevalence of Morquio syndrome, type A is estimated at 1 in 169,000 [2]. 

Another aspect of the linkage of keratan sulfate to protein also deserves attention, i.e., the macromolecular structure of the proteoglycan. It has been indicated previously that skeletal keratan sulfate is linked to the... 

Caterson, B., J.E. Christner, and J.R. Baker. 1983. Identification of a monoclonal antibody that specifically recognizes comeal and skeletal keratan sulfate. J. Biol Chem. 258 8848-8854. 

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