Mucopolysaccharides

Mucopolysaccharides minangka rantai panjang molekul gula sing ditemokake ing awak, asring ing lendir lan cairan ing sekitar sendi. Dheweke umume diarani glycosaminoglycans.

Nalika awak ora bisa ngrusak mucopolysaccharides, kedadeyan sing diarani mucopolysaccharidoses (MPS). MPS nuduhake klompok kelainan metabolisme sing diwarisake. Wong sing duwe MPS ora duwe, utawa cukup, zat (enzim) sing dibutuhake kanggo ngrusak rantai molekul gula.

Wangun MPS kalebu:

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

Glycosaminoglycans; GAG

Kumar V, Abbas AK, Aster JC. Kelainan genetik. Ing: Kumar V, Abbas AK, Aster JC, eds. Robbins lan Basis Penyakit Patologis Cotran. Edhisi kaping 9 Philadelphia, PA: Elsevier Saunders; 2015: bab 5.

Pyeritz RE. Penyakit warisan saka jaringan ikat. Ing: Goldman L, Schafer AI, eds. Pangobatan Goldman-Cecil. Edhisi kaping 26 Philadelphia, PA: Elsevier; 2020: bab 244.

Spranger JW. Mucopolysaccharidoses. Ing: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Buku Teks Pediatrik Nelson. Edhisi 21 Philadelphia, PA: Elsevier; 2020: bab 107.