CD2-associated protein (CD2AP) expression in podocytes rescues lethality of CD2AP deficiency
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CD2-associated protein (CD2AP) expression in podocytes rescues lethality of CD2AP deficiency. / Grunkemeyer, James A; Kwoh, Christopher; Huber, Tobias B; Shaw, Andrey S.
In: J BIOL CHEM, Vol. 280, No. 33, 19.08.2005, p. 29677-81.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - CD2-associated protein (CD2AP) expression in podocytes rescues lethality of CD2AP deficiency
AU - Grunkemeyer, James A
AU - Kwoh, Christopher
AU - Huber, Tobias B
AU - Shaw, Andrey S
PY - 2005/8/19
Y1 - 2005/8/19
N2 - Mice born without CD2-associated protein (CD2AP) develop renal failure and nephrotic syndrome about 4 weeks after birth and die around 6 weeks of age. Although CD2AP is widely expressed, the severity of the renal failure precludes a clear determination of the role of CD2AP in other tissues. Here we generated transgenic mice expressing CD2AP using a podocyte-specific promoter. Podocyte-specific expression of CD2AP prevented the development of proteinuria, demonstrating that the renal failure is solely due to loss of CD2AP in podocytes and not in other renal or in immune cells. CD2AP-deficient mice are long-lived and appear phenotypically normal. Histological analysis demonstrated testicular abnormalities that were age-related. CIN85, a paralog of CD2AP, is poorly expressed in both the podocyte and the basal seminiferous tubule, suggesting that the loss of CD2AP in specific tissues may be compensated for by CIN85.
AB - Mice born without CD2-associated protein (CD2AP) develop renal failure and nephrotic syndrome about 4 weeks after birth and die around 6 weeks of age. Although CD2AP is widely expressed, the severity of the renal failure precludes a clear determination of the role of CD2AP in other tissues. Here we generated transgenic mice expressing CD2AP using a podocyte-specific promoter. Podocyte-specific expression of CD2AP prevented the development of proteinuria, demonstrating that the renal failure is solely due to loss of CD2AP in podocytes and not in other renal or in immune cells. CD2AP-deficient mice are long-lived and appear phenotypically normal. Histological analysis demonstrated testicular abnormalities that were age-related. CIN85, a paralog of CD2AP, is poorly expressed in both the podocyte and the basal seminiferous tubule, suggesting that the loss of CD2AP in specific tissues may be compensated for by CIN85.
KW - Adaptor Proteins, Signal Transducing
KW - Animals
KW - Cytoskeletal Proteins
KW - Epithelial Cells
KW - Infertility, Male
KW - Kidney Glomerulus
KW - Male
KW - Mice
KW - Mice, Transgenic
KW - Phenotype
KW - Proteins
KW - Renal Insufficiency
KW - Testis
KW - Journal Article
U2 - 10.1074/jbc.M504004200
DO - 10.1074/jbc.M504004200
M3 - SCORING: Journal article
C2 - 15951437
VL - 280
SP - 29677
EP - 29681
JO - J BIOL CHEM
JF - J BIOL CHEM
SN - 0021-9258
IS - 33
ER -