keratan sulfate


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keratan sulfate

 [ker´ah-tan]
either of two sulfated mucopolysaccharides (I and II). Keratan sulfate is an important component of the proteoglycan of cartilage, and occurs in the cornea and the nucleus pulposus, and is also an accumulation product in Morquio's syndrome.

ker·a·tan sul·fate

(ker'ă-tan sŭl'fāt),
A type of sulfated mucopolysaccharide containing d-galactose in place of the uronic acid of hyaluronic acid or chondroitin; also containing unsulfated and 6-sulfated N-acetyl-d-glucosamine; found in cartilage, bone, connective tissue, the cornea, aorta, and in the intervertebral disks; accumulates in Morquio syndrome; keratan sulfate I is abundant in cornea and is attached to a protein via an asparaginyl residue; keratan sulfate II is found in loose connective tissue and bone and is linked to a seryl or threonyl residue.
Synonym(s): keratosulfate

keratan sulfate

(kĕr′ə-tăn′)
n.
Any of several glycosaminoglycans found especially in the cornea, cartilage, and bone.

ker·a·tan sul·fate

(ker'ă-tan sŭl'fāt)
A type of sulfated mucopolysaccharide found in cartilage, bone, connective tissue, the cornea, aortae, and in the intervertebral discs; accumulates in Morquio syndrome.
Synonym(s): keratosulfate.
References in periodicals archive ?
Recently, the use of an immune-based assay for the quantification of keratan sulfate in MPS IVA has been reported (49).
Identification of a common mutation in mucopolysaccharidosis IVA: correlation among genotype, phenotype, and keratan sulfate. J Hum Genet 2004;49:490-4.
The CHST6 gene encodes human GlcNAc-6-O-sulfotransferase (EC 2.8.2.-) that transfers sulfate to the 6-O position of GlcNAc and Gal residues in the poly-N-acetyllactosamine extensions in keratan sulfate. GlcNAc-6-O-sulfotransferase is produced in human cornea, brain, spinal cord, and trachea.
Keratan sulfate in serum and cartilage is nonsulfated or low-sulfated (107, 108).
Currently, MCD can be diagnosed with an ELISA that makes use of a monoclonal antibody specific for a sulfated epitope on keratan sulfate (110).
Absence of normal keratan sulfate in the blood of patients with macular corneal dystrophy.
Macular dystrophy of the cornea: a systemic disorder of keratan sulfate metabolism.
In addition, the secondary objectives are to evaluate urinary keratan sulfate (KS) levels and growth velocity, stated the company.
For example, keratan sulfate, the major stored substrate in MPS IVA, is not produced in large amounts by skin fibroblasts and hence is not stored.
These disorders all store either heparan sulfate, dermatan sulfate, keratan sulfate, or a combination of these.
(d) Keratan sulfate. (e) Multiple sulfatase deficiency.