Mucopolysaccharides

Mucopolysaccharides mangrupikeun ranté panjang molekul gula anu aya di sakujur awak, sering di lendir sareng dina cairan sakitar sendi. Aranjeunna langkung sering disebat glycosaminoglycans.

Nalika awak henteu tiasa ngarecah mucopolysaccharides, kaayaan anu disebut mucopolysaccharidoses (MPS) lumangsung. MPS ngarujuk kana kelompok gangguan warisan metabolisme. Jalma anu ngagaduhan MPS henteu ngagaduhan, atanapi cekap, tina zat (énzim) anu diperyogikeun pikeun ngarecah ranté molekul gula.

Bentuk MPS kalebet:

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

Glycosaminoglycans; GAG

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Pyeritz RE. Panyakit warisan tina jaringan konéktip. Di: Goldman L, Schafer AI, eds. Pangobatan Goldman-Cecil. Kaping 26 ed. Philadelphia, PA: Elsevier; 2020: bab 244.

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