The glomerular filtration barrier: a structural target for novel kidney therapies

IS Daehn, JS Duffield - Nature Reviews Drug Discovery, 2021 - nature.com
Loss of normal kidney function affects more than 10% of the population and contributes to
morbidity and mortality. Kidney diseases are currently treated with immunosuppressive …

Regulation of the actin cytoskeleton in podocytes

J Blaine, J Dylewski - Cells, 2020 - mdpi.com
Podocytes are an integral part of the glomerular filtration barrier, a structure that prevents
filtration of large proteins and macromolecules into the urine. Podocyte function is …

INF2 mutations cause kidney disease through a gain-of-function mechanism

B Subramanian, S Williams, S Karp, MF Hennino… - Science …, 2024 - science.org
Heterozygosity for inverted formin-2 (INF2) mutations causes focal segmental
glomerulosclerosis (FSGS) with or without Charcot-Marie-Tooth disease. A key question is …

The formin INF2 in disease: progress from 10 years of research

L Labat-de-Hoz, MA Alonso - Cellular and Molecular Life Sciences, 2020 - Springer
Formins are a conserved family of proteins that primarily act to form linear polymers of actin.
Despite their importance to the normal functioning of the cytoskeleton, for a long time, the …

[HTML][HTML] Altered endoplasmic reticulum integrity and organelle interactions in living cells expressing INF2 variants

QTH Tran, N Kondo, H Ueda, Y Matsuo… - International Journal of …, 2024 - mdpi.com
The cytoskeleton mediates fundamental cellular processes by organizing inter-organelle
interactions. Pathogenic variants of inverted formin 2 (INF2) CAAX isoform, an actin …

Podocyte infolding glomerulopathy: a case series report and literature review

Y Feng, W Wang, Y Zou, T Chen, W Wang, G Li… - Journal of Clinical …, 2023 - mdpi.com
Background: Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare
manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical …

Regulation of formin INF2 and its alteration in INF2-linked inherited disorders

L Labat-de-Hoz, MÁ Jiménez, I Correas… - Cellular and Molecular …, 2024 - Springer
Formins are proteins that catalyze the formation of linear filaments made of actin. INF2, a
formin, is crucial for correct vesicular transport, microtubule stability and mitochondrial …

Dysregulated dynein-mediated trafficking of nephrin causes INF2-related podocytopathy

H Sun, C Perez-Gill, JS Schlöndorff… - Journal of the …, 2021 - journals.lww.com
Background FSGS caused by mutations in INF2 is characterized by a podocytopathy with
mistrafficked nephrin, an essential component of the slit diaphragm. Because INF2 is a …

Synaptopodin deficiency exacerbates kidney disease in a mouse model of Alport syndrome

L Ning, HY Suleiman, JH Miner - American Journal of …, 2021 - journals.physiology.org
Synaptopodin (Synpo) is an actin-associated protein in podocyte foot processes. By
generating mice that completely lack Synpo, we previously showed that Synpo is …

[HTML][HTML] Drebrin protects assembled actin from INF2-FFC-mediated severing and stabilizes cell protrusions

S Rajan, R Aguirre, ZH Zhou, P Hauser… - Journal of Molecular …, 2024 - Elsevier
Highly specialized cells, such as neurons and podocytes, have arborized morphologies that
serve their specific functions. Actin cytoskeleton and its associated proteins are responsible …