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Volumn 11, Issue 3, 2001, Pages 322-327

Congenital nephrotic syndromes

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA ACTININ; F ACTIN; NEPHRIN; PODOCIN; PROTEIN; UNCLASSIFIED DRUG;

EID: 0035370260     PISSN: 0959437X     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0959-437X(00)00197-0     Document Type: Review
Times cited : (30)

References (35)
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    • The authors describe the generation of nephrin antibodies by which they can, for the first time, localize nephrin at the glomerular filtration barrier. In immunoelectron microscopy, nephrin is found at the slit between podocyte foot processes. A zipper-like model for nephrin assembly in the slit diaphragm is presented.
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    • Using homologous recombination, a mouse model for congenital nephrotic syndrome (NPHS1) is generated by inactivating the nephrin gene in embryonic stem cells with a lacZ gene reporter construct. The knock-out mice develop massive proteinuria and die within 24 hours. The kidneys of null mice exhibit enlarged Bowman's spaces, dilated tubuli, effacement of podocyte foot processes and absence of the slit diaphragm, essentially as found in human NPHS1 patients. In addition to glomerular podocytes expression, the reporter gene is also expressed in the brain and pancreas. Expression in the brain is restricted to the ventricular zone of the fourth ventricle, the developing spinal cord, cerebellum, hippocampus and olfactory bulb. In the cerebellum, the expression is seen in radial glial cells.
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* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.