The serine protease hepsin mediates urinary secretion and polymerisation of Zona Pellucida domain protein uromodulin

M Brunati, S Perucca, L Han, A Cattaneo, F Consolato… - Elife, 2015 - elifesciences.org
Uromodulin is the most abundant protein in the urine. It is exclusively produced by renal
epithelial cells and it plays key roles in kidney function and disease. Uromodulin mainly …

Analysis of uromodulin polymerization provides new insights into the mechanisms regulating ZP domain-mediated protein assembly

C Schaeffer, S Santambrogio, S Perucca… - Molecular biology of …, 2009 - Am Soc Cell Biol
Uromodulin is the most abundant protein secreted in urine, in which it is found as a high-
molecular-weight polymer. Polymerization occurs via its zona pellucida (ZP) domain, a …

Urinary uromodulin carries an intact ZP domain generated by a conserved C-terminal proteolytic cleavage

S Santambrogio, A Cattaneo, I Bernascone… - Biochemical and …, 2008 - Elsevier
Uromodulin (or Tamm–Horsfall protein) is the most abundant protein in human urine under
physiological conditions. Little is known about the molecular mechanism of uromodulin …

A structured interdomain linker directs self-polymerization of human uromodulin

M Bokhove, K Nishimura, M Brunati… - Proceedings of the …, 2016 - National Acad Sciences
Uromodulin (UMOD)/Tamm–Horsfall protein, the most abundant human urinary protein,
plays a key role in chronic kidney diseases and is a promising therapeutic target for …

Cryo‐EM structure of native human uromodulin, a zona pellucida module polymer

A Stsiapanava, C Xu, M Brunati… - The EMBO …, 2020 - embopress.org
Assembly of extracellular filaments and matrices mediating fundamental biological
processes such as morphogenesis, hearing, fertilization, and antibacterial defense is driven …

Evolving concepts in uromodulin biology, physiology, and its role in disease: a tale of two forms

KA LaFavers, R Micanovic, AR Sabo, LA Maghak… - …, 2022 - Am Heart Assoc
Uromodulin (or Tamm-Horsfall protein) is a glycoprotein uniquely produced in the kidney by
tubular cells of the thick ascending limb of the loop of Henle and early distal tubules. This …

Mutant uromodulin expression leads to altered homeostasis of the endoplasmic reticulum and activates the unfolded protein response

C Schaeffer, S Merella, E Pasqualetto, D Lazarevic… - PLoS …, 2017 - journals.plos.org
Uromodulin is the most abundant urinary protein in physiological conditions. It is exclusively
produced by renal epithelial cells lining the thick ascending limb of Henle's loop (TAL) and it …

Defective intracellular trafficking of uromodulin mutant isoforms

I Bernascone, S Vavassori, A Di Pentima… - Traffic, 2006 - Wiley Online Library
Medullary cystic kidney disease/familial juvenile hyperuricemic nephropathy (MCKD/FJHN)
are autosomal dominant renal disorders characterized by tubulo‐interstitial fibrosis …

Uromodulin (Tamm–Horsfall protein): Guardian of urinary and systemic homeostasis

R Micanovic, K LaFavers, PS Garimella… - Nephrology Dialysis …, 2020 - academic.oup.com
Biology has taught us that a protein as abundantly made and conserved among species as
Tamm–Horsfall protein (THP or uromodulin) cannot just be a waste product serving no …

The cryo-EM structure of the human uromodulin filament core reveals a unique assembly mechanism

JJ Stanisich, DS Zyla, P Afanasyev, J Xu, A Kipp… - Elife, 2020 - elifesciences.org
The glycoprotein uromodulin (UMOD) is the most abundant protein in human urine and
forms filamentous homopolymers that encapsulate and aggregate uropathogens, promoting …