Umlobi: Clyde Lopez
Usuku Lokudalwa: 19 Ujulayi 2021
Ukuvuselela Usuku: 1 Epreli 2025
Anonim
Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes
Ividiyo: Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes

AmaMucopolysaccharides ngamaketanga amade amangqamuzana kashukela atholakala emzimbeni wonke, imvamisa ku-mucus nakuluketshezi oluzungeze amalunga. Zibizwa kakhulu ngama-glycosaminoglycans.

Lapho umzimba ungakwazi ukwehlisa i-mucopolysaccharides, kwenzeka isimo esibizwa nge-mucopolysaccharidoses (MPS). I-MPS ibhekisa eqenjini lokuphazamiseka okuzuzwe njengefa komzimba. Abantu abane-MPS abanakho, noma okwanele, kwento (i-enzyme) edingekayo ukudiliza amaketanga e-molecule yoshukela.

Amafomu ama-MPS afaka:

  • I-MPS I (i-Hurler syndrome; i-Hurler-Scheie syndrome; i-Scheie syndrome)
  • I-MPS II (Hunter syndrome)
  • I-MPS III (iSanfilippo syndrome)
  • I-MPS IV (iMorquio syndrome)

AmaGlycosaminoglycans; IGAG

UKumar V, Abbas AK, Aster JC. Izinkinga zofuzo. Ku: Kumar V, Abbas AK, Aster JC, ama-eds. AmaRobbins kanye neCotran Pathologic Basis of Disease. Umhlaka 9. IPhiladelphia, PA: Elsevier Saunders; 2015: isahluko 5.

UPyeritz RE. Izifo ezizuze njengefa zezicubu ezixhuma. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 244.


I-Spranger JW. I-Mucopolysaccharidoses. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 107.

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