期刊
CELLULAR AND MOLECULAR LIFE SCIENCES
卷 67, 期 6, 页码 891-906出版社
BIRKHAUSER VERLAG AG
DOI: 10.1007/s00018-009-0228-7
关键词
Keratan sulphate; Proteoglycan; Glycosaminoglycan; Cornea; Collagen
资金
- BBSRC [BB/D001919/1]
- NIH/NEI [EY014620]
- MRC Programme [G0600755]
- EPSRC [EP/F034970/1]
- MRC [G0600755] Funding Source: UKRI
- Biotechnology and Biological Sciences Research Council [BB/D001919/1, B18021, BBS/B/10994] Funding Source: researchfish
- Engineering and Physical Sciences Research Council [EP/F034970/1] Funding Source: researchfish
- Medical Research Council [G0600755] Funding Source: researchfish
- NATIONAL EYE INSTITUTE [R01EY014620] Funding Source: NIH RePORTER
Keratan sulphate (KS) is the predominant glycosaminoglycan (GAG) in the cornea of the eye, where it exists in proteoglycan (PG) form. KS-PGs have long been thought to play a pivotal role in the establishment and maintenance of the array of regularly-spaced and uniformly-thin collagen fibrils which make up the corneal stroma. This characteristic arrangement of fibrils allows light to pass through the cornea. Indeed, perturbations to the synthesis of KS-PG core proteins in genetically altered mice lead to structural matrix alterations and corneal opacification. Similarly, mutations in enzymes responsible for the sulphation of KS-GAG chains are causative for the inherited human disease, macular corneal dystrophy, which is manifested clinically by progressive corneal cloudiness starting in young adulthood.
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