Results 31 to 40 of about 16,635 (234)

Regulating complement in the kidney: insights from CFHR5 nephropathy

open access: yesDisease Models & Mechanisms, 2011
Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role
Daniel P. Gale, Matthew C. Pickering
doaj   +1 more source

Complement-Mediated Glomerular Diseases: A Tale of 3 Pathways

open access: yesKidney International Reports, 2016
A renewed interest in the role of complement in the pathogenesis of glomerular diseases has improved our understanding of their basic, underlying physiology.
Andrew S. Bomback   +2 more
doaj   +1 more source

Recurrent C3 Glomerulonephritis with an ADAMTS 13 Gene Variant: A Case Report and Literature Review

open access: yesSaudi Journal of Kidney Diseases and Transplantation, 2021
C3 glomerulonephritis (C3GN) is a recently described form of GN that mainly occurs in children and young adults. It results from dysregulation of the alternative complement pathway. Studies have shown that dense deposit disease has a high recurrence rate;
Reem A. Al Zahrani   +2 more
doaj   +1 more source

Gain of function mutant of complement factor B K323E mimics pathogenic C3NeF autoantibodies in convertase assays

open access: yesAutoimmunity, 2018
Complement convertases are enzymatic complexes, which play a critical role in propagation and amplification of the complement cascade. Under physiological conditions, convertases decay shortly after being formed in either spontaneous or inhibitor-driven ...
Aleksandra Urban   +6 more
doaj   +1 more source

Kayser fleischer ring: A strong clinical indicator of neuro-wilson's

open access: yesTNOA Journal of Ophthalmic Science and Research, 2017
A female child presenting with features of lupus nephritis was referred for ophthalmic evaluation related to autoimmune diseases. It turned out to be a dense yellow-brown deposit of Kayser Fleischer (KF) Ring and sunflower cataract following which ...
Rajalakshmi Selvaraj   +2 more
doaj   +1 more source

C3 Glomerulopathy: A Review with Emphasis on Ultrastructural Features

open access: yesGlomerular Diseases, 2022
C3 glomerulopathy (C3G) is a rare disease resulting from dysregulation of the alternative complement pathway, resulting in the deposition of complement component 3 (C3) in the kidney.
Jean Hou, Kevin Yi Mi Ren, Mark Haas
doaj   +1 more source

Modulation of Homer1 EVH1 domain internal dynamics by putative autism‐associated mutations

open access: yesFEBS Letters, EarlyView.
The putative autism‐associated M65I and S97L variants of the EVH1 domain of the postsynaptic scaffold protein Homer1 do not exhibit substantial changes in their overall structure or partner binding. Both of them, but especially the M65I variant, show altered internal dynamics relative to the wild‐type domain on the μs‐ms timescale, indicated by the ...
Fanni Farkas   +6 more
wiley   +1 more source

From mice to humans—divergent strategies for intestinal homeostasis and regeneration

open access: yesFEBS Letters, EarlyView.
Recent advances such as organoid genome editing, xenotransplantation, imaging, and whole‐genome sequencing have enabled direct studies of human intestinal stem cells (ISCs). These studies reveal species‐specific features, including slower ISC proliferation, distinct injury responses, slower somatic mutation accumulation in humans, and an inverse ...
Keiko Ishikawa   +2 more
wiley   +1 more source

Treatment of C3 Glomerulopathy in Adult Kidney Transplant Recipients: A Systematic Review

open access: yesMedical Sciences, 2020
Background: C3 glomerulopathy (C3G), a rare glomerular disease mediated by alternative complement pathway dysregulation, is associated with a high rate of recurrence and graft loss after kidney transplantation (KTx).
Maria L Gonzalez Suarez   +11 more
doaj   +1 more source

Diagnostic Problems in C3 Glomerulopathy

open access: yesBiomedicines, 2023
Background: C3 glomerulopathies (C3GN) are a group of rare kidney diseases associated with impaired complement regulation. The effects of this disease include the accumulation of complement C3 in the kidneys. Based on the clinical data, as well as light,
Leszek Niepolski   +11 more
doaj   +1 more source

Home - About - Disclaimer - Privacy