ライフサイエンスコーパス: podocin

1 d desmin, and a recovery of synaptopodin and podocin.

2 foot process junctional markers nephrin and podocin.

3 Mec2, which encodes the homolog of mammalian Podocin.

4 h reduced urinary levels of both nephrin and podocin.

5 e presence of aquaporin-2, polycystin-1, and podocin.

6 Podocin accumulates in an oligomeric form in lipid rafts

7 mplicated in the etiology of FSGS, including podocin, alpha-actinin-4, CD2-associated protein (CD2AP)

8 ragm-associated proteins (including nephrin, podocin, alpha-actinin-4, CD2-associated protein, and tr

9 this regard, the human mutations in nephrin, podocin, alpha-actinin-4, COL4A3, and COL4A5 genes expre

10 les (and a non-human serum control), nephrin podocin and CD2AP assumed a cytoplasmic distribution; ne

11 n vitro studies reveal direct interaction of podocin and CD2AP.

12 ormal expression levels of podocyte markers, podocin and desmin.

13 ate an intimate relationship between nephrin podocin and filamentous actin, and reason that disruptio

14 ail vein injection using the kidney-specific podocin and gamma-glutamyl transferase promoters, but fo

15 Podocin and its Caenorhabditis elegans orthologue MEC-2

16 Although podocin and MEC-2 are membrane-associated proteins with

17 al regulators that control the expression of podocin and nephrin and thereby mediate podocyte differe

18 nd desmin gene expression were increased and podocin and nephrin content were decreased by either the

19 tial peak of proteinuria and podocyte mRNAs (podocin and nephrin) followed 8 d later by a second peak

20 ficant reduction of slit membrane molecules (podocin and nephrin), key GBM components (fibronectin, l

21 It colocalizes with podocin and regulates its stability.

22 modulate expression of Nphs2, which encodes podocin and several other podocyte-expressed genes.

23 -4 in podocytes, whereas the localization of podocin and synaptopodin remained relatively intact.

24 annel activation depended on cholesterol and podocin and was inhibited by stabilization of the actin

25 regulatory regions of both CD2AP and NPHS2 (podocin) and demonstrated that LMX1B binds to these sequ

26 on the slit diaphragm (SD) proteins nephrin, podocin, and CD2-associated protein (CD2AP) to function

27 stituting molecules (SDCM), such as nephrin, podocin, and CD2-associated protein (CD2AP), were decrea

28 oth unique membrane proteins (e.g., nephrin, podocin, and Neph1) and typical adherens junction protei

29 sses the specific podocyte proteins nephrin, podocin, and synaptopodin were examined by patch clamp.

30 cyte markers Wilms tumor protein 1, nephrin, podocin, and synaptopodin.

31 ferentiation markers (synaptopodin, nephrin, podocin, and WT-1) in HIV-1-infected podocytes.

32 aricalcitol preserved expression of nephrin, podocin, and WT1; prevented proteinuria; and reduced glo

33 ne, the subcellular distribution of nephrin, podocins, and CD2AP and their functional interaction wit

34 PHB2 coprecipitated with podocin, another SPFH domain-containing protein, essenti

35 lar injury associated with increased urinary podocin:aquaporin 2 and nephrin:aquaporin 2 molar ratios

36 lso known as KIRREL), CD2AP, ZO-1 (TJP1) and podocin, are expressed in the nephrocyte and form a comp

37 Mutations in NPHS2, the gene that encodes podocin, are well-established causes of both familial an

38 eover, GST pull-down experiments reveal that podocin associates via its COOH-terminal domain with CD2

39 that the FF diet decreased the expression of podocin but increased desmin and ceramide levels in glom

40 pression of the podocyte markers nephrin and podocin, but there was no loss of cells.

41 r genes NPHS1 and NPHS2 encoding nephrin and podocin cause two types of severe nephrotic syndrome pre

42 plasma leads to a concentration of nephrin, podocin, CD2AP, and actin at the cell surface.

43 luding the novel podocyte proteins, nephrin, podocin, CD2AP, and synaptopodin, and known molecules of

44 ns in the same protein complexes as nephrin, podocin, CD2AP, ZO-1, and Neph1 by cosedimentation, coim

45 tations, with sequence variants, and with no podocin changes could not be distinguished from each oth

46 The tracking of single cells in podocin-confetti mice featuring cell-specific expression

47 omology domain of the slit diaphragm protein podocin contained one such site, threonine 234 (T234), l

48 actin, and reason that disruption of nephrin/podocin could be a final common pathway leading to foot

49 The kidneys of podocin-Cre beta1-fl/fl mice exhibit normal glomerular e

50 odocyte specific deletion of integrin beta1 (podocin-Cre beta1-fl/fl mice) are born normal but cannot

51 ated mice deficient of ILK in the podocytes (podocin-Cre ILK-fl/fl mice).

52 beta1(flox/flox) mice with podocyte specific podocin-cre mice (pod-Cre), which express cre at the tim

53 Expression of nephrin, podocin, desmin, and transient receptor potential channe

54 tions revealed a role for T234 in regulating podocin dimerization.

55 The T234 site resides at the interface of podocin dimers.

56 clampsia associated with an elevated urinary podocin(+) EVs-to-nephrin(+) EVs ratio and may be mediat

57 in-positive to nephrin-positive urinary EVs (podocin(+) EVs-to-nephrin(+) EVs ratio) and increased ne

58 atively correlated with proteinuria, urinary podocin(+) EVs-to-nephrin(+) EVs ratio, and nephrinuria.

59 derm development, as assessed by nephrin and podocin expression in double osr1/sox32-deficient embryo

60 Ultrastructurally, disruption of nephrin or podocin expression resulted in a loss of slit-diaphragms

61 Moreover, mutant podocin failed to activate the ion channel TRPC6, which

62 Mutations in the human podocin gene NPHS2 cause familial or sporadic forms of r

63 ESRD on 9p21.3, 1q25.1 (in the region of the podocin gene), and 13q33.3.

64 .5-kb fragment derived from the human NPHS2 (podocin) gene was designed in a similar fashion to drive

65 In podocin-GFP mice, podocytes formed sporadic multicellula

66 increased the expression of synaptopodin and podocin, improved podocyte viability, and reduced the mi

67 ndidate regions for nephrin, CD2AP, WT1, and podocin in this sample.

68 subsets of glomeruli with perturbed nephrin, podocin, integrin alpha3 and fibronectin expression.

69 quitin ligase Ubr4 is a key component of the podocin interactome purified both from cultured podocyte

70 That podocin interacts with CD2AP and nephrin in vivo is show

71 The NPHS2 gene product podocin is a key component of the slit diaphragm cell ju

72 Podocin is a key protein of the kidney podocyte slit dia

73 Podocin is expressed in glomerular podocytes, but its su

74 the erythrocyte lipid raft protein stomatin, podocin is present in high-order oligomers and may serve

75 ic analysis of mouse glomeruli revealed that podocin is ubiquitylated at two lysine residues.

76 Its product, podocin, is a new member of the stomatin family, which c

77 podocyte-specific protein, nephrin, but not podocin, is reduced in preeclamptic compared with normot

78 we show, by immunoelectron microscopy, that podocin localizes to the podocyte foot process membrane,

79 phrin), CD2AP, Wilms tumor (WT1), and NPHS2 (podocin) loci.

80 The PHB-domain protein podocin maintains the renal filtration barrier and its m

81 6 (TRPC6) interacting with the MEC-2 homolog podocin may form a mechanosensitive ion channel complex

82 lation of one site, K301, do not only target podocin/MEC-2 for proteasomal degradation, but may also

83 y demonstrates that the carboxyl terminus of podocin/MEC-2 has to be placed at the inner leaflet of t

84 tored podocyte loss by detecting nephrin and podocin mRNA in urine particulates with quantitative rev

85 In older age podocyte detachment rate (urine podocin mRNA-to-creatinine ratio) was higher than at you

86 of a genotype-phenotype correlation between podocin mutations and age of onset, a worldwide cohort o

87 stant nephrotic syndrome, which is caused by podocin mutations in about 25% of children and nearly 15

88 ngenital nephrotic syndrome with nephrin and podocin mutations resulting from triallelic mutations re

89 Recessive podocin mutations were present in 18.1% (73 of 404) of f

90 han any other patient group, with or without podocin mutations, in this study (mean onset >4.17 yr).

91 podocin mutations, with single heterozygous podocin mutations, with sequence variants, and with no p

92 Patient groups with other recessive podocin mutations, with single heterozygous podocin muta

93 is significantly earlier than for any other podocin mutations.

94 Like Nephrin and Podocin, Neph1 was enriched in Triton X-100 detergent-re

95 on of the slit diaphragm-associated proteins podocin, nephrin, and synaptopodin and to enhanced trans

96 ortant podocyte proteins, including those of podocin, nephrin, neph1, alpha-actinin-4, and vimentin.

97 Mutations in the gene encoding podocin (NPHS2) cause autosomal recessive steroid-resist

98 We used the human podocin (NPHS2) gene promoter to control expression of t

99 ier revealed that absence of normal nephrin, podocin or mosaic eyes expression results in loss of glo

100 A similar complex with the podocin ortholog MEC-2 is required for touch sensation i

101 the mechanosensory complex that requires the podocin ortholog MEC2 for assembly.

102 Podocin(P118L) and MEC-2(P134S) did not fractionate in d

103 tation that causes kidney disease in humans (podocin(P118L)) has also been identified in C. elegans i

104 lymerization studies showed that nephrin and podocin partially co-localize with actin, most strikingl

105 of proteinuria and podocyte mRNAs that were podocin positive but nephrin negative.

106 vesicles, which colocalized with megalin, in podocin-positive cells.

107 amptic pregnancies contained a high ratio of podocin-positive to nephrin-positive urinary EVs (podoci

108 ia that persisted for months correlated with podocin-positive, nephrin-negative mRNAs in urine.

109 nvestigate this question, we used the 2.5 kb podocin promoter to target Flag-tagged human vitamin D r

110 t in HIFalpha hyperstabilization, we crossed podocin promoter-Cre transgenic mice, which express Cre

111 Cre-mediated recombination controlled by the podocin promoter.

112 tes under the control of the zebrafish nphs2/podocin promoter.

113 t negative AA-4E-BP1 transgene driven by the podocin promoter; a member of the mammalian target of ra

114 in ectatic tubules expressed podocalyxin and podocin proteins but not nephrin, compatible with detach

115 sion and altered localization of nephrin and podocin proteins.

116 Whereas podocin resides at a specialized cell junction at the po

117 Dual transgenic mice, bearing both podocin-rtTA and tetO-LacZ transgenes, had no detectable

118 On doxycycline induction, podocin-rtTA:tet-O-VEGF164 mice express twofold higher k

119 We postulate that podocin serves in the structural organization of the sli

120 O-1 and alpha-actinin-4, whereas nephrin and podocin solubility were unchanged.

121 ation did not initially affect expression of podocin, synaptopodin, and nephrin but reduced their exp

122 ng mutations of proteins such as nephrin and podocin that are expressed at or near the podocyte slit

123 nephritic syndrome of the Finnish type, and Podocin, the gene mutated in autosomal recessive steroid

124 , claudin-1 interacted with both nephrin and podocin through cis- and trans-associations in cultured

125 and podocytes were stressed (increased urine podocin-to-nephrin mRNA ratio).

126 n of the slit diaphragm proteins nephrin and podocin was decreased, and expression of the transcripti

127 nding to the promoter regions of nephrin and podocin was increased in RA-treated podocytes.

128 addition to membrane expression, nephrin and podocin were detected intracellularly in a filamentous p

129 podocyte proteins synaptopodin, nephrin, and podocin were expressed normally.

130 wever, mRNA and protein levels for CD2AP and podocin were greatly reduced, suggesting a cooperative r

131 Zebrafish nephrin and podocin were specifically expressed in pronephric podocy

132 , including WT-1, synaptopodin, nephrin, and podocin, were not expressed by any cells in glomerular c

133 The discovery of the genes for nephrin and podocin, which are mutated in two types of congenital ne