Differential muscular glycogen clearance after enzyme replacement therapy in a mouse model of Pompe disease

Glycogen storage disease in the α-glucosidase knockout6neo(−)/6neo(−) (GAA KO) mouse, a model of Pompe disease, results in the pathologic accumulation of glycogen primarily within skeletal myocytes and cardiomyocytes. Intravenous administration of recombinant human α-glucosidase (rhGAA, Myozyme®, ag...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Molecular genetics and metabolism 2007-08, Vol.91 (4), p.343-351
Hauptverfasser:	Hawes, Michael L., Kennedy, William, O’Callaghan, Michael W., Thurberg, Beth L.
Format:	Artikel
Sprache:	eng
Schlagworte:	Aglucosidase alfa alpha-Glucosidases - deficiency alpha-Glucosidases - genetics alpha-Glucosidases - therapeutic use Animals Capillaries - enzymology Capillaries - physiopathology Disease Models, Animal Fiber type Glycogen Glycogen Storage Disease Type II - enzymology Glycogen Storage Disease Type II - pathology Glycogen Storage Disease Type II - therapy Humans Lysosome Mice Mice, Knockout Muscle Muscle, Skeletal - blood supply Muscle, Skeletal - enzymology Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Myozyme Pompe disease Twitch α-Glucosidase
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Schreiben Sie den ersten Kommentar!