Search Results - "van den Heuvel, Lambertus W P J"
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Levamisole Modulation of Podocytes’ Actin Cytoskeleton in Nephrotic Syndrome
Published in Biomedicines (01-11-2023)“…Podocytes play a central role in glomerular diseases such as (idiopathic) nephrotic syndrome (iNS). Glucocorticoids are the gold standard therapy for iNS…”
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The role of properdin in complement-mediated renal diseases: a new player in complement-inhibiting therapy?
Published in Pediatric nephrology (Berlin, West) (01-08-2019)“…Properdin is known as the only positive regulator of the complement system. Properdin promotes the activity of this defense system by stabilizing its key…”
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Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency
Published in American Journal of Physiology: Cell Physiology (01-10-2005)“…Complex I (NADH:ubiquinone oxidoreductase) is the largest multisubunit assembly of the oxidative phosphorylation system, and its malfunction is associated with…”
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Shiga Toxin Selectively Upregulates Expression of Syndecan-4 and Adhesion Molecule ICAM-1 in Human Glomerular Microvascular Endothelium
Published in Toxins (03-07-2020)“…Hemolytic uremic syndrome (HUS) is a severe renal disease that is often preceded by infection with Shiga toxin (Stx)-producing (STEC). The exact mechanism of…”
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Transferrin mutations at the glycosylation site complicate diagnosis of congenital disorders of glycosylation type I
Published in Journal of inherited metabolic disease (01-08-2011)“…Congenital disorders of glycosylation (CDG) form a group of metabolic disorders caused by deficient glycosylation of proteins and/or lipids. Isoelectric…”
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Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort
Published in Pediatric nephrology (Berlin, West) (01-03-2022)“…Background C3 glomerulopathy (C3G) is a rare kidney disorder characterized by predominant glomerular depositions of complement C3. C3G can be subdivided into…”
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Case Report: Variable Pharmacokinetic Profile of Eculizumab in an aHUS Patient
Published in Frontiers in immunology (15-01-2021)“…With the introduction of eculizumab, a C5-inhibitor, morbidity and mortality improved significantly for patients with atypical hemolytic uremic syndrome…”
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Whole Exome Sequencing in Patients with the Cuticular Drusen Subtype of Age-Related Macular Degeneration
Published in PloS one (23-03-2016)“…Age-related macular degeneration (AMD) is the leading cause of irreversible blindness in elderly people worldwide. Cuticular drusen (CD) is a clinical subtype…”
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Different Aspects of Classical Pathway Overactivation in Patients With C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis
Published in Frontiers in immunology (11-08-2021)“…The rare and heterogeneous kidney disorder C3 glomerulopathy (C3G) is characterized by dysregulation of the alternative pathway (AP) of the complement system…”
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Glyco-iELISA: a highly sensitive and unambiguous serological method to diagnose STEC-HUS caused by serotype O157
Published in Pediatric nephrology (Berlin, West) (01-04-2019)“…Background Providing proof of presence of Shiga toxin–producing E. coli (STEC) infection forms the basis for differentiating STEC-hemolytic uremic syndrome…”
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Ca2+-mobilizing agonists increase mitochondrial ATP production to accelerate cytosolic Ca2+ removal: aberrations in human complex I deficiency
Published in American Journal of Physiology: Cell Physiology (01-08-2006)“…Previously, we reported that both the bradykinin (Bk)-induced increase in mitochondrial ATP concentration ([ATP]M) and the rate of cytosolic Ca2+ removal are…”
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Overactivity of Alternative Pathway Convertases in Patients With Complement-Mediated Renal Diseases
Published in Frontiers in immunology (04-04-2018)“…Overactivation of the alternative pathway of the complement system is associated with the renal diseases atypical hemolytic uremic syndrome (aHUS) and C3…”
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Novel Assays to Distinguish Between Properdin-Dependent and Properdin-Independent C3 Nephritic Factors Provide Insight Into Properdin-Inhibiting Therapy
Published in Frontiers in immunology (17-06-2019)“…C3 glomerulopathy (C3G) is an umbrella classification for severe renal diseases characterized by predominant staining for complement component C3 in the…”
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Lack of specific binding of Shiga-like toxin (verocytotoxin) and non-specific interaction of Shiga-like toxin 2 antibody with human polymorphonuclear leucocytes
Published in Nephrology, dialysis, transplantation (01-03-2007)“…Background. After gastrointestinal infection with Shiga-like toxin (Stx) producing Escherichia coli, the toxin is transported from the intestine to the renal…”
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Mutations in the chloride channel gene CLCNKB as a cause of classic Bartter syndrome
Published in Journal of the American Society of Nephrology (01-08-2000)“…Inherited hypokalemic renal tubulopathies are differentiated into at least three clinical subtypes: (1) the Gitelman variant of Bartter syndrome (GS); (2)…”
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Functional expression of mutations in the human NaCl cotransporter: Evidence for impaired routing mechanisms in gitelman's syndrome
Published in Journal of the American Society of Nephrology (01-06-2002)“…Gitelman's syndrome is an autosomal recessive renal tubular disorder characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria. This…”
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Detection of verocytotoxin bound to circulating polymorphonuclear leukocytes of patients with hemolytic uremic syndrome
Published in Journal of the American Society of Nephrology (01-04-2001)“…The epidemic form of hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children and is characterized by a prodromal phase of…”
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Plasma exchange or immunoadsorption for recurrent focal segmental glomerulosclerosis: clear differences in vitro
Published in Nephrology, dialysis, transplantation (19-10-2022)Get full text
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Proteinuria and Exposure to Eculizumab in Atypical Hemolytic Uremic Syndrome
Published in Clinical journal of the American Society of Nephrology (01-06-2023)“…Eculizumab is a monoclonal antibody for the treatment of atypical hemolytic uremic syndrome (aHUS). Kidney damage, a common condition in patients with aHUS,…”
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Interactions of Shiga-like toxin with human peripheral blood monocytes
Published in Pediatric nephrology (Berlin, West) (01-08-2007)“…The cytotoxic effect of Shiga-like toxin (Stx; produced by certain Escherichia coli strains) plays a central role in typical hemolytic uremic syndrome (HUS)…”
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