Analysis Tools
mortality/aging
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• mice exhibit a reduced life span with a median age at death of 9 months
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homeostasis/metabolism
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• significant increase in serum creatinine levels at 6 months of age, indicating renal dysfunction
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• mice develop persistent proteinuria by 3 months of age
• rate of proteinuria increases modestly until 6 months and accelerates more rapidly thereafter
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• after induction of kidney podocyte injury by protamine sulfate (PS) perfusion, mutant foot processes exhibit a broadened and flattened morphology that is distinct from that in wild-type controls
• PS-treated mutant podocytes develop unusually long fine processes that project from primary, secondary, and tertiary processes, suggesting pseudovillous transformation
• PS-treated mutant podocytes fail to recover normal morphology following subsequent infusion of heparin sulfate, unlike wild-type controls
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renal/urinary system
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• mice exhibit no evidence of glomerular or interstitial scarring, even at 9 months of age
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• mice develop persistent proteinuria by 3 months of age
• rate of proteinuria increases modestly until 6 months and accelerates more rapidly thereafter
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• mice exhibit evidence of fine finger-like projections from the podocyte cell bodies and processes at 6 months of age
• foot process spreading is evident by 8 months of age
• following PS perfusion, foot processes exhibit a broadened and flattened morphology that is distinct from that in wild-type controls
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• PS-treated podocytes develop unusually long fine processes that project from primary, secondary, and tertiary processes, suggesting pseudovillous transformation
• PS-treated mutant podocytes fail to recover normal morphology following subsequent infusion of heparin sulfate, unlike wild-type controls
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integument
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• mice exhibit a scruffed appearance at 9 months of age
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