Podocin
Mostrando 1-12 de 13 artigos, teses e dissertações.
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1. Expressão gênica de moléculas associadas ao podócito em pacientes portadores de glomerulopatias proteinúricas
Introdução: A injúria ao podócito glomerular tem um papel crítico para o surgimento de proteinúria em diferentes glomerulopatias. Neste estudo avaliamos a expressão gênica das proteínas associadas ao podócito em biópsias renais e na urina simultaneamente em pacientes com glomerulopatias proliferativas (GPP) e não proliferativas (GPNP) proteinúri
IBICT - Instituto Brasileiro de Informação em Ciência e Tecnologia. Publicado em: 2012
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2. Podocin, a raft-associated component of the glomerular slit diaphragm, interacts with CD2AP and nephrin
NPHS2 was recently identified as a gene whose mutations cause autosomal recessive steroid-resistant nephrotic syndrome. Its product, podocin, is a new member of the stomatin family, which consists of hairpin-like integral membrane proteins with intracellular NH2- and COOH-termini. Podocin is expressed in glomerular podocytes, but its subcellular distribution
American Society for Clinical Investigation.
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3. Podocin Inactivation in Mature Kidneys Causes Focal Segmental Glomerulosclerosis and Nephrotic Syndrome
Podocin is a critical component of the glomerular slit diaphragm, and genetic mutations lead to both familial and sporadic forms of steroid-resistant nephrotic syndrome. In mice, constitutive absence of podocin leads to rapidly progressive renal disease characterized by mesangiolysis and/or mesangial sclerosis and nephrotic syndrome. Using established Cre-lo
American Society of Nephrology.
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4. Urine Podocyte mRNAs Mark Progression of Renal Disease
Because loss of podocytes associates with glomerulosclerosis, monitoring podocyte loss by measuring podocyte products in urine may be clinically useful. To determine whether a single episode of podocyte injury would cause persistent podocyte loss, we induced limited podocyte depletion using a diphtheria toxin receptor (hDTR) transgenic rat. We monitored podo
American Society of Nephrology.
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5. Early Glomerular Filtration Defect and Severe Renal Disease in Podocin-Deficient Mice
Podocytes are specialized epithelial cells covering the basement membrane of the glomerulus in the kidney. The molecular mechanisms underlying the role of podocytes in glomerular filtration are still largely unknown. We generated podocin-deficient (Nphs2−/−) mice to investigate the function of podocin, a protein expressed at the insertion of the slit dia
American Society for Microbiology.
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6. The LIM-homeodomain transcription factor Lmx1b plays a crucial role in podocytes
Patients with nail-patella syndrome often suffer from a nephropathy, which ultimately results in chronic renal failure. The finding that this disease is caused by mutations in the transcription factor LMX1B, which in the kidney is expressed exclusively in podocytes, offers the opportunity for a better understanding of the renal pathogenesis. In our analysis
American Society for Clinical Investigation.
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7. NPHS2 mutations in late-onset focal segmental glomerulosclerosis: R229Q is a common disease-associated allele
Mutations in NPHS2, encoding podocin, have been identified in childhood onset focal and segmental glomerulosclerosis (FSGS). The role of NPHS2 in adult disease is less well defined. We studied 30 families with FSGS and apparent autosomal recessive inheritance and 91 individuals with primary FSGS. We screened family members for NPHS2 mutations. NPHS2 mutation
American Society for Clinical Investigation.
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8. Nephrin and CD2AP Associate with Phosphoinositide 3-OH Kinase and Stimulate AKT-Dependent Signaling
Mutations of NPHS1 or NPHS2, the genes encoding nephrin and podocin, as well as the targeted disruption of CD2-associated protein (CD2AP), lead to heavy proteinuria, suggesting that all three proteins are essential for the integrity of glomerular podocytes, the visceral glomerular epithelial cells of the kidney. It has been speculated that these proteins par
American Society for Microbiology.
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9. Slit Diaphragms Contain Tight Junction Proteins
Slit diaphragms are essential components of the glomerular filtration apparatus, as changes in these junctions are the hallmark of proteinuric diseases. Slit diaphragms, considered specialized adherens junctions, contain both unique membrane proteins (e.g., nephrin, podocin, and Neph1) and typical adherens junction proteins (e.g., P-cadherin, FAT, and cateni
American Society of Nephrology.
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10. Clinical Features and Long-Term Outcome of Nephrotic Syndrome Associated with Heterozygous NPHS1 and NPHS2 Mutations
Background and objectives: Mutations in nephrin (NPHS1) and podocin (NPHS2) genes represent a major cause of idiopathic nephrotic syndrome (NS) in children. It is not yet clear whether the presence of a single mutation acts as a modifier of the clinical course of NS.
American Society of Nephrology.
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11. Transcriptional induction of slit diaphragm genes by Lmx1b is required in podocyte differentiation
LMX1B encodes a LIM-homeodomain transcription factor. Mutations in LMX1B cause nail-patella syndrome (NPS), an autosomal dominant disease with skeletal abnormalities, nail hypoplasia, and nephropathy. Expression of glomerular basement membrane (GBM) collagens is reduced in Lmx1b–/– mice, suggesting one basis for NPS nephropathy. Here, we show that Lmx1b�
American Society for Clinical Investigation.
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12. Unusual Thermal Disassembly of the SPFH Domain Oligomer from Pyrococcus horikoshii
Stomatin, prohibitin, flotillin, and HflK/C (SPFH) domain proteins are membrane proteins that are widely conserved from bacteria to mammals. The molecular functions of these proteins have not been established. In mammals, the domain is often found in raft-associated proteins such as flotillin and podocin. We determined the structure of the SPFH domain of PH0
The Biophysical Society.