Onkọwe Ọkunrin: Clyde Lopez
ỌJọ Ti ẸDa: 19 OṣU Keje 2021
ỌJọ ImudojuiwọN: 19 OṣU Keje 2025
Anonim
Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes
Fidio: Mucopolysaccharide Storage Disease Type I: Hurler, Hurler-Scheie, and Scheie syndromes

Mucopolysaccharides jẹ awọn ẹwọn gigun ti awọn molikula suga ti o wa ni gbogbo ara, nigbagbogbo ni imu ati ninu omi ni ayika awọn isẹpo. Wọn pe wọn julọ wọpọ glycosaminoglycans.

Nigbati ara ko ba le fọ mucopolysaccharides lulẹ, ipo ti a pe ni mucopolysaccharidoses (MPS) waye. MPS tọka si ẹgbẹ kan ti awọn rudurudu ti a jogun ti iṣelọpọ. Awọn eniyan ti o ni MPS ko ni eyikeyi, tabi to, ti nkan kan (enzymu) ti o nilo lati fọ awọn ẹwọn molikula suga.

Awọn fọọmu ti MPS pẹlu:

  • MPS I (Arun Hurler; Aarun Hurler-Scheie; Aarun Scheie)
  • MPS II (Hunter dídùn)
  • MPS III (Sanfilippo dídùn)
  • MPS IV (Morquio dídùn)

Glycosaminoglycans; GAG

Kumar V, Abbas AK, Aster JC. Awọn rudurudu Jiini. Ni: Kumar V, Abbas AK, Aster JC, awọn eds. Robbins ati Ipilẹ Pathologic Cotran ti Arun. 9th ed. Philadelphia, PA: Elsevier Saunders; 2015: ori 5.

Pyeritz RE. Awọn arun ti a jogun ti ẹya ara asopọ. Ni: Goldman L, Schafer AI, awọn eds. Oogun Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: ori 244.


Spranger JW. Mucopolysaccharidoses. Ni: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Iwe-ẹkọ ti Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: ori 107.

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