Number of Nephrons in Normal Human Kidneys and Kidneys of Patients with the Congenital Nephrotic Syndrome

Tryggvason, Karl; Kouvalainen, K

doi:10.1159/000180493

Cited by 35 publications

(20 citation statements)

References 11 publications

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“…The number per kidney is 0.88 x 106 in the control kidneys, but as high as 1.53 x 10ft in the CNF kidneys, a difference of 74%. This agrees with the pre vious study in which the sieving method was used, and in which the mean glomerular number in four CNF kidneys was shown to be about 70% higher than in seven control kid neys [6].…”

Section: Resultssupporting

confidence: 91%

“…The disease is inherited as an autosomal recessive trait [3] with an exceptionally high incidence in Finland, 12.4:100.000 births [4], Histological examination shows glomerular changes like mesangial hypercellularity, scler osis of the capillary tuft and fibrosis, and the proximal tubuli are often ectatically dilated with epithelial cell atrophy [2,5]. Recently, it has been shown that the number of glomeruli is about 70% higher in kidneys from patients with CNF than in normal kidneys, being 3.2* I0'1 and l.8*IO fi, respectively, when using a sieving method for the counting of glomeruli [6],…”

Section: Introductionmentioning

confidence: 99%

“…The first is a statistico-geo metrical method which depends on the count ing of glomeruli in a series of sections taken from a given volume of renal tissue. The second is based on the resistance of the Bowman's capsule to trauma when a given volume of renal tissue is ground and suspended in a given volume of saline and the glomeruli are then counted in a counting cell [7], The sieving pro cedure [6] makes use of the latter principle.…”

Section: Introductionmentioning

confidence: 99%

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Morphometric Studies on Glomeruli in the Congenital Nephrotic Syndrome

Tryggvason¹

1978

Nephron

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Morphometric studies were performed on glomeruli in two kidneys of a 13-month-old patient with congenital nephrotic syndrome of the Finnish type (CNF) and two control kidneys from an 11-month-old child. The mean diameter of the glomeruli in the diseased kidneys was abnormally large for the patient’s age and the mean glomerular volume about twice the normal. The number of glomeruli, counted using a statistico-geometrical method, was 74% higher than in the control kidneys, a finding which is in accordance with a previous result obtained using a different counting method. The total glomerular volume per kidney in the CNF patient was about 3 times the normal for that age, almost equalling that in a 7-year-old individual.

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Section: Resultssupporting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Morphometric Studies on Glomeruli in the Congenital Nephrotic Syndrome

Tryggvason¹

1978

Nephron

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“…The glomeruli were isolated from 8-to 12-wk-old adult mice and from human cadaver kidneys that were unsuitable for transplantation (from the IV Department of Surgery of Helsinki, Finland). The isolation methods have been described previously (23,27). The Western blotting was done following standard procedures using polyvinyl difluoride membrane and horseradish peroxidase-conjugated secondary antibody (Amersham Biosciences).…”

Section: Western Blottingmentioning

confidence: 99%

Expression and Subcellular Distribution of Novel Glomerulus-Associated Proteins Dendrin, Ehd3, Sh2d4a, Plekhh2, and 2310066E14Rik

Patrakka¹,

Xiao²,

Nukui³

et al. 2007

Journal of the American Society of Nephrology

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The glomerular capillary tuft is a highly specialized microcapillary that is dedicated to function as a sophisticated molecular sieve. The glomerulus filter has a unique molecular composition, and several essential glomerular proteins are expressed in the kidney exclusively by glomerular podocytes. A catalog of >300 glomerulus-upregulated transcripts that were identified using expressed sequence tag profiling and microarray analysis was published recently. This study characterized the expression profile of five glomerulus-upregulated transcripts/proteins (ehd3, dendrin, sh2d4a, plekhh2, and 2310066E14Rik) in detail. The expression pattern of these novel glomerular transcripts in various mouse tissues was studied using reverse transcriptase-PCR, Northern blotting, and in situ hybridization. For studying the distribution of corresponding proteins, polyclonal antibodies were raised against the gene products, and Western blotting, immunofluorescence, and immunoelectron microscopic analyses were performed. Remarkably, it was discovered that all five transcripts/proteins were expressed in the kidney exclusively by glomerular cells. Ehd3 was expressed only by glomerular endothelial cells. Importantly, ehd3 is the first gene ever shown to be expressed exclusively by glomerular endothelial cells and not by other endothelial cells in the kidney. Dendrin, sh2d4a, plekhh2, and 2310066E14Rik, however, were transcribed solely by podocytes. With the use of polyclonal antibodies, dendrin, sh2d4a, and plekhh2 proteins were localized to the slit diaphragm and the foot process, whereas 2310066E14Rik protein was localized to the podocyte major processes and cell body. This study provides fresh insights into glomerular biology and uncovers new possibilities to explore the role of these novel proteins in the glomerular physiology and pathology. T he glomerular capillary tuft of the kidney is a unique micro-organ that is specialized to operate as a sophisticated molecular sieve (1). The filtration barrier is composed of the glomerular endothelial cells (GEC), the glomerular basement membrane (GBM), and the podocyte cells. GEC differ from most other endothelial cells in that they are extraordinarily flattened and fenestrated (2). The fenestrated structure of GEC depends on podocyte-derived vascular endothelial growth factor A (3), but the mechanisms behind their unique structure largely are unknown. The GBM, like all basement membranes (BM), is composed of interconnected collagen type IV and laminin networks, where proteoglycans and other matrix components are attached. The GBM, however, is distinct from most other BM of the body, because it contains a unique composition of collagen type IV and laminin isoforms. The importance of these components is highlighted by the fact that defects in GBM-specific type IV collagen or laminin lead to Alport syndrome and Pierson syndrome, respectively (4 -6).The podocyte foot processes and the slit diaphragm serve as a final filtration barrier in the glomerulus. This filtration machinery contains a n...

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“…The isolation of kidney glomeruli was performed by sieving as described [17]. Isolated glomeruli were homogenized with Ultra-Turrax (Rose Scientific Ltd, Alberta, Canada) in 0.08 M Tris-HCl pH 6.8 buffer containing 2% SDS and 10% glycerol.…”

Section: Western Blottingmentioning

confidence: 99%

Glomerular endothelium in kidneys with congenital nephrotic syndrome of the Finnish type (NPHS1)

Kaukinen

Kuusniemi

Lautenschlager

et al. 2007

Nephrology Dialysis Transplantation

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Background. The role of glomerular capillary endothelium in the pathophysiology of nephrotic kidney diseases is poorly known. We analysed the glomerular endothelial lesions in kidneys from patients with congenital nephrotic syndrome of the Finnish type (NPHS1). The disorder is caused by a genetic defect in a major podocyte slit diaphragm protein, nephrin. It manifests as nephrotic syndrome soon after birth and leads to glomerular sclerosis in early childhood. Methods. The glomerular capillary and endothelial cell lesions in NPHS1 kidneys nephrectomized at infancy were studied by electron and light microscopy, immunohistochemistry and cytokine antibody array. Results. Mesangial expansion and capillary obliteration were evident in practically all NPHS1 glomeruli. No thrombus formation was detected by fibrin staining. Electron microscopy revealed endothelial blebs (endotheliosis). The endothelial fenestration and the attachment of endothelial cells to the basement membrane were, however, quite normal. This fits to the abundant expression of a vascular endothelial growth factor (VEGF) and its transcription factor, hypoxia-inducible factor-1α (HIF-1α), in NPHS1 glomeruli. The proliferative activity of the intracapillary cells was modest and no apoptosis was detected. The expression of an endothelial adhesion molecule, intercellular adhesion molecule 1 (ICAM-1) and several chemokines was upregulated in NPHS1 glomeruli as compared to adult control kidneys. The recruitment of leukocytes carrying ligands for the major endothelial adhesion molecules, however, was modest in the mesangial area of NPHS1 glomeruli. Conclusions. The findings indicate that the glomerular endothelium is quite resistant to the nephrotic state in NPHS1 kidneys and underscores the importance of mesangial cells in the progression of glomerular sclerosis.

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Number of Nephrons in Normal Human Kidneys and Kidneys of Patients with the Congenital Nephrotic Syndrome

Cited by 35 publications

References 11 publications

Morphometric Studies on Glomeruli in the Congenital Nephrotic Syndrome

Morphometric Studies on Glomeruli in the Congenital Nephrotic Syndrome

Expression and Subcellular Distribution of Novel Glomerulus-Associated Proteins Dendrin, Ehd3, Sh2d4a, Plekhh2, and 2310066E14Rik

Glomerular endothelium in kidneys with congenital nephrotic syndrome of the Finnish type (NPHS1)

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