Lysosomal Lipid Storage Disease from the Perspective of General Pediatricians | Zendy

Beyhan Özkaya | Zendy; Ebru Canda | Zendy; Sema Kalkan Uçar | Zendy

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Lysosomal Lipid Storage Disease from the Perspective of General Pediatricians

Author(s) -

Beyhan Özkaya,

Ebru Canda,

Sema Kalkan Uçar

Publication year - 2016

Publication title -

the journal of pediatric research

Language(s) - Turkish

Resource type - Journals

eISSN - 2587-2478

pISSN - 2147-9445

DOI - 10.4274/jpr.43650

Subject(s) - medicine , perspective (graphical) , disease , intensive care medicine , family medicine , pathology , visual arts , art

Lizozomlar hucre icinde mukopolisakkaridler, sfingolipidler, glikoproteinler, trigliserid ve kolesterol esterleri gibi urunlerin yikimini saglayan hidrolitik enzimleri iceren asidik yapili organellerdir. Lizozomal hastaliklarin etyopatogenezi iki ana grupta toplanabilir. Ilk grupta, lizozomal enzimin eksikligi, o enzime ozgu substratin birikimi, buna bagli olarak da hucrenin zarar gormesi ve islevinin bozulmasi gerceklesir. Ikinci grupta ise lizozomlarda hidrolize olmus molekullerin hucre disina tasinmasini saglayan transport protein kusurlarina bagli patolojik birikim gozlenir. Lizozomal depo hastaliklarinda (LDH) klinik bulgular; depolanan maddeye ve organizmadaki dagilimina gore farklilik gosterir (1). Ayni hastalikta degisik

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