Mucopolysaccharides

Tha mucopolysaccharides nan slabhraidhean fada de mholacilean siùcair a lorgar air feadh a ’chuirp, gu tric ann am mucus agus ann an sruthadh timcheall nan joints. Mar as trice canar glycosaminoglycans riutha.

Nuair nach urrainn don bhodhaig mucopolysaccharides a bhriseadh sìos, tha suidheachadh ris an canar mucopolysaccharidoses (MPS) a ’tachairt. Tha MPS a ’toirt iomradh air buidheann de dhuilgheadasan dìleab metabolism. Chan eil susbaint (no gu leòr) de stuth (enzyme) aig daoine le MPS a dh ’fheumar gus na slabhraidhean moileciuil siùcair a bhriseadh sìos.

Tha foirmean MPS a ’toirt a-steach:

• MPS I (Syndrome Hurler; Syndrome Hurler-Scheie; syndrome Scheie)
• MPS II (syndrome Hunter)
• MPS III (syndrome Sanfilippo)
• MPS IV (syndrome Morquio)

Glycosaminoglycans; GAG

Kumar V, Abbas AK, Aster JC. Eas-òrdughan ginteil. Ann an: Kumar V, Abbas AK, Aster JC, eds. Bunait Galar Robbins agus Cotran Pathologic. 9mh deas. Philadelphia, PA: Elsevier Saunders; 2015: caib 5.

Pyeritz RE. Galaran oighreachail de stuth-ceangail. Ann an: Goldman L, Schafer AI, eds. Leigheas Goldman-Cecil. 26mh deas. Philadelphia, PA: Elsevier; 2020: caib 244.

Spranger JW. Mucopolysaccharidoses. Ann an: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Leabhar-teacsa Nelson de Pediatrics. 21mh deas. Philadelphia, PA: Elsevier; 2020: caib 107.