[HTML][HTML] Receptor-mediated endocytosis of α-galactosidase A in human podocytes in Fabry disease

T Prabakaran, R Nielsen, JV Larsen, SS Sørensen… - PloS one, 2011 - journals.plos.org
… and podocyte repair is an important therapeutic target. In Fabry disease, podocyte injury
is … This study identifies in the human podocyte three endocytic receptors, mannose 6-phosphate/…

[PDF][PDF] Receptor-Mediated Endocytosis of α-Galactosidase A in Human Podocytes in Fabry

T Prabakaran, R Nielsen, U Feldt-Rasmussen… - 2011 - academia.edu
Fabry disease, podocyte injury is caused by the intracellular accumulation of
globotriaosylceramide. This study identifies in the human podocyte three endocytic … in the …

[HTML][HTML] Mannose 6-phosphate receptor and sortilin mediated endocytosis of α-galactosidase A in kidney endothelial cells

T Prabakaran, R Nielsen, SC Satchell, PW Mathieson… - PloS one, 2012 - journals.plos.org
… Hence, the effectiveness of treatment with ERT in Fabry disease needs further clarification
… in the receptor-mediated uptake of recombinant α-Gal A in human podocytes and HEK293 …

Signaling pathways in cell models of Fabry disease nephropathy

A Labilloy - 2015 - d-scholarship.pitt.edu
Fabry disease and control immortalized human podocytes using a high-throughput
phosphorylation microarray. Fabry disease podocytes … the lysosomal hydrolase α -galactosidase A …

[HTML][HTML] Lysosomal delivery of therapeutic enzymes in cell models of Fabry disease

D Marchesan, TM Cox, PB Deegan - … of inherited metabolic disease, 2012 - Springer
… of α -Galactosidase A into cells relevant to Fabry disease, in … α -Galactosidase A uptake was
examined in fibroblasts, four … by storage in Fabry disease (cardiomyocytes, renal podocytes, …

[HTML][HTML] Mannose receptor-mediated delivery of moss-made α-galactosidase A efficiently corrects enzyme deficiency in Fabry mice

JS Shen, A Busch, TS Day, XL Meng, CI Yu… - … metabolic disease, 2016 - Springer
… in Fabry disease, a glycosphingolipidosis caused by deficient activity of α-galactosidase A
(α-… Cardiomyocytes, podocytes, and peripheral neurons represent the major hard-to-reach cell …

[HTML][HTML] Molecular basis for globotriaosylceramide regulation and enzyme uptake in immortalized aortic endothelial cells from Fabry mice

XL Meng, TS Day, N McNeill, P Ashcraft… - … metabolic disease, 2016 - Springer
Fabry disease is caused by deficient activity of α-galactosidase A and subsequent …
moss-aGal into FMEC2 was via receptor-mediated endocytosis or non-specific pinocytosis, …

Distribution of α-galactosidase A in normal human kidney and renal accumulation and distribution of recombinant α-galactosidase A in Fabry mice

EI Christensen, Q Zhou, SS Sørensen… - Journal of the …, 2007 - journals.lww.com
… Deficiency of lysosomal α-galactosidase A (α-Gal A) in Fabry disease results in cellular … is
taken up by podocytes and reabsorbed by receptor-mediated endocytosis in proximal tubule …

[HTML][HTML] Dysregulated autophagy contributes to podocyte damage in Fabry's disease

MC Liebau, F Braun, K Höpker, C Weitbrecht, V Bartels… - PloS one, 2013 - journals.plos.org
… in α-galactosidase A-deficient podocytes may be the result of deficient mTOR kinase activity.
This finding links the lysosomal enzymatic defect in Fabry’s disease … of Fabry's disease we …

[HTML][HTML] Fabry disease: molecular basis, pathophysiology, diagnostics and potential therapeutic directions

K Kok, KC Zwiers, RG Boot, HS Overkleeft, JMFG Aerts… - Biomolecules, 2021 - mdpi.com
Fabry disease (FD) is a lysosomal storage disorder (LSD) characterized by the deficiency of
α-galactosidase A (… Of note, in a human podocyte cell line, three endocytic receptors, IGF2R/…