[HTML][HTML] Megalin and cubilin in proximal tubule protein reabsorption: from experimental models to human disease

R Nielsen, EI Christensen, H Birn - Kidney international, 2016 - Elsevier
Proximal tubule protein uptake is mediated by 2 receptors, megalin and cubilin. These
receptors rescue a variety of filtered ligands, including biomarkers, essential vitamins, and …

Membranous nephropathy

P Ronco, L Beck, H Debiec, FC Fervenza… - Nature reviews Disease …, 2021 - nature.com
Membranous nephropathy (MN) is a glomerular disease that can occur at all ages. In adults,
it is the most frequent cause of nephrotic syndrome. In~ 80% of patients, there is no …

Developments in the treatment of Fabry disease

SJ van der Veen, CEM Hollak… - Journal of inherited …, 2020 - Wiley Online Library
Enzyme replacement therapy (ERT) with recombinant α‐galactosidase A (r‐αGAL A) for the
treatment of Fabry disease has been available for over 15 years. Long‐term treatment may …

PGRN acts as a novel regulator of mitochondrial homeostasis by facilitating mitophagy and mitochondrial biogenesis to prevent podocyte injury in diabetic …

D Zhou, M Zhou, Z Wang, Y Fu, M Jia, X Wang… - Cell death & …, 2019 - nature.com
Mitochondrial dysfunction is considered as a key mediator in the pathogenesis of diabetic
nephropathy (DN). Therapeutic strategies targeting mitochondrial dysfunction hold …

Endocytic receptors in the renal proximal tubule

EI Christensen, H Birn, T Storm, K Weyer… - Physiology, 2012 - journals.physiology.org
Protein reabsorption is a predominant feature of the renal proximal tubule. Animal studies
show that the ability to rescue plasma proteins relies on the endocytic receptors megalin and …

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 (α-GalA) and the consequent accumulation of toxic metabolites such as …

Agalsidase benefits renal histology in young patients with Fabry disease

C Tøndel, L Bostad, KK Larsen, A Hirth… - Journal of the …, 2013 - journals.lww.com
The effect of early-onset enzyme replacement therapy on renal morphologic features in
Fabry disease is largely unknown. Here, we evaluated the effect of 5 years of treatment with …

Fabry disease therapy: state-of-the-art and current challenges

O Azevedo, MF Gago, G Miltenberger-Miltenyi… - International journal of …, 2020 - mdpi.com
Fabry disease (FD) is a lysosomal storage disorder caused by mutations of the GLA gene
that lead to a deficiency of the enzymatic activity of α-galactosidase A. Available therapies …

[HTML][HTML] Membranous nephropathy: a review on the pathogenesis, diagnosis, and treatment

WL Lai, TH Yeh, PM Chen, CK Chan… - Journal of the Formosan …, 2015 - Elsevier
In adults, membranous nephropathy (MN) is a major cause of nephrotic syndrome. However,
the etiology of approximately 75% of MN cases is idiopathic. Secondary causes of MN are …

Effects of enzyme replacement therapy and antidrug antibodies in patients with Fabry disease

M Lenders, E Brand - Journal of the American Society of …, 2018 - journals.lww.com
Fabry disease (FD) is an X-linked lysosomal storage disorder (LSD) caused by mutations of
the a-galactosidase A gene. The lysosomal enzyme a-galactosidase A (GLA) mediates the …