Journal: medRxiv

Article Title: GIPC1 intermediate-length repeat expansion in amyotrophic lateral sclerosis

doi: 10.1101/2025.05.22.25328088

Figure Lengend Snippet: (A) The CGG repeat is located within exon 1 of GIPC1 . Primers and probes for repeat-primed PCR (RP-PCR, green), fragment analysis (blue), and RNA fluorescence in situ hybridization (FISH, red) were designed within or adjacent to the repeat region. (B) The left panel shows the results for RP-PCR, in which abnormal CGG repeat expansions were detected in the upper three cases. The middle panel displays the results of fragment analysis. The right panel presents histograms of repeat sizes estimated from long-read sequencing data using NanoRepeat; orange bars indicate expanded alleles. (C) Histogram of CGG repeat sizes determined through fragment analysis. The lower graph shows an enlarged view of the low-frequency range. In the control group, most alleles contained 32 or fewer repeats. Two control samples exhibited abnormally expanded alleles, whereas four ALS samples harbored alleles with 33 or more repeats.

Article Snippet: GIPC1 immunostaining of spinal cord sections from patients with ALS (A) Anterior horn of the lumbar spinal cord from an ALS patient with a CGG repeat expansion stained with anti-GIPC1 antibody (Proteintech). (B) Anterior horn of the lumbar spinal cord from an ALS patient without a CGG repeat expansion stained with anti-GIPC1 antibody (Proteintech). (C) Anterior horn of the lumbar spinal cord from an ALS patient with a CGG repeat expansion stained with anti-GIPC1 antibody (Santa Cruz Biotechnology). (D) Anterior horn of the lumbar spinal cord from an ALS patient without a CGG repeat expansion stained with anti-GIPC1 antibody (Santa Cruz Biotechnology).

Techniques: Fluorescence, In Situ Hybridization, Sequencing, Control

Journal: medRxiv

Article Title: GIPC1 intermediate-length repeat expansion in amyotrophic lateral sclerosis

doi: 10.1101/2025.05.22.25328088

Figure Lengend Snippet: (A) Results of RNA FISH using a (CGG) 8 probe. Intranuclear RNA foci were observed exclusively in the ALS patient harboring a CGG repeat expansion in GIPC1 . (B) RNA FISH results using another probe targeting the 5′ UTR of GIPC1 . Similar intranuclear RNA foci were detected with this GIPC1 -specific probe, confirming the presence of expanded repeat-containing RNA. Numbers in parentheses indicate the number of CGG repeats in GIPC1 . Scale bars: 5 μm.

Article Snippet: GIPC1 immunostaining of spinal cord sections from patients with ALS (A) Anterior horn of the lumbar spinal cord from an ALS patient with a CGG repeat expansion stained with anti-GIPC1 antibody (Proteintech). (B) Anterior horn of the lumbar spinal cord from an ALS patient without a CGG repeat expansion stained with anti-GIPC1 antibody (Proteintech). (C) Anterior horn of the lumbar spinal cord from an ALS patient with a CGG repeat expansion stained with anti-GIPC1 antibody (Santa Cruz Biotechnology). (D) Anterior horn of the lumbar spinal cord from an ALS patient without a CGG repeat expansion stained with anti-GIPC1 antibody (Santa Cruz Biotechnology).

Techniques:

Journal: Biomedicines

Article Title: Immunoexpression Patterns of Megalin, Cubilin, Caveolin-1, Gipc1 and Dab2IP in the Embryonic and Postnatal Development of the Kidneys in Yotari ( Dab1 −/− ) Mice

doi: 10.3390/biomedicines12071542

Figure Lengend Snippet: Megalin, Cubilin, Caveolin-1, Gipc1 and Dab2IP are receptors of ligands in kidney structures. In proximal tubular epithelial cells, Megalin, with its cytoplasmic tail, makes a signal for clathrin-mediated endocytosis. Cubilin must join with Megalin or Amnionless (AMN) for successful signaling. After uptake, ligands are relieved in vesicles and transported to the other structures of the cell, while receptors are returned to the apical membrane . Caveolin-1 is the major structure of caveolae in the plasma membrane, which is crucial for cellular metabolism and, consequently, for kidney diseases . Gipc1 interacts with lots of transmembrane receptors (for example, GPCR) for intercellular signaling . Dab2IP is a signaling adaptor that binds with inflammatory cytokines and growth factors . Created with BioRender.com .

Article Snippet: , Gipc1 Polyclonal antibody , 14822-1-AP , Rabbit , 1:100 , Proteintech Group, Inc., Rosemont, IL, USA.

Techniques: Membrane, Clinical Proteomics

Journal: Biomedicines

Article Title: Immunoexpression Patterns of Megalin, Cubilin, Caveolin-1, Gipc1 and Dab2IP in the Embryonic and Postnatal Development of the Kidneys in Yotari ( Dab1 −/− ) Mice

doi: 10.3390/biomedicines12071542

Figure Lengend Snippet: Antibodies used for immunofluorescence.

Article Snippet: , Gipc1 Polyclonal antibody , 14822-1-AP , Rabbit , 1:100 , Proteintech Group, Inc., Rosemont, IL, USA.

Techniques: Immunofluorescence, Labeling, Plasmid Preparation

Journal: Biomedicines

Article Title: Immunoexpression Patterns of Megalin, Cubilin, Caveolin-1, Gipc1 and Dab2IP in the Embryonic and Postnatal Development of the Kidneys in Yotari ( Dab1 −/− ) Mice

doi: 10.3390/biomedicines12071542

Figure Lengend Snippet: Immunofluorescence staining of embryonic wild-type and yotari mouse kidneys was performed using the Gipc1 ( a – d ) marker. Arrows indicate the immunoexpression patterns of Gipc1 in the metanephric mesenchyme (mm), glomeruli (g), renal vesicles (rv), ampullae (A), convoluted tubules (Ct) and collecting ducts (Cd), as shown on the 4′,6-diamidino-2-phenylindole (DAPI) nuclei staining image. Immunoexpression of Gipc1, DAPI staining and the merged images of Gipc1 and DAPI was observed at embryonic days E13.5 and E15.5 in wild-type ( a , c ) and yotari ( b , d ) kidneys. Images were taken at ×40 magnification, and the scale bar represents 100 μm for all images. The percentages of Gipc1 ( e ) positive cells in the metanephric mesenchyme (mm), renal vesicles (rv) or glomeruli (g), convoluted tubules (Ct) and ampullae (A) or collecting ducts (Cd) of wild-type and yotari kidneys at embryonic days 13.5 and 15.5 are shown. Data are presented as the mean ± SD (vertical line) and analyzed using a two-way ANOVA followed by Tukey’s multiple comparison test. Significant differences are indicated by **** p < 0.0001. Ten substructures were assessed at each time point.

Article Snippet: , Gipc1 Polyclonal antibody , 14822-1-AP , Rabbit , 1:100 , Proteintech Group, Inc., Rosemont, IL, USA.

Techniques: Immunofluorescence, Staining, Marker, Comparison

Journal: Biomedicines

Article Title: Immunoexpression Patterns of Megalin, Cubilin, Caveolin-1, Gipc1 and Dab2IP in the Embryonic and Postnatal Development of the Kidneys in Yotari ( Dab1 −/− ) Mice

doi: 10.3390/biomedicines12071542

Figure Lengend Snippet: Immunofluorescence staining of postnatal wild-type and yotari mouse kidneys was performed using the Gipc1 ( a ) marker. Arrows show the immunoexpression patterns of Gipc1 in glomeruli (g), distal convoluted tubules (dct) and proximal convoluted tubules (pct), indicated on 4′,6-diamidino-2-phenylindole (DAPI) nuclei staining image. Immunoexpression of Gipc1 staining and merged Gipc1 and DAPI at 14 days (P14) in wild-type mice. Wild-type and yotari mice at postnatal days 4 and 14 of kidney development predominantly matched the localization and intensity of Caveolin-1. Therefore, representative images were taken from wild-type mice. Images were taken at ×40 magnification, and the scale bar represents 100 μm for all images. The percentages of Gipc1 positive cells ( b ) positive cells in the glomeruli (G), distal convoluted tubules (DCT) and proximal convoluted tubules (PCT) of postnatal kidneys of wild type and yotari animals over time (P4 and P14). Data are presented as the mean ± SD (vertical line) and analyzed using a two-way ANOVA followed by Tukey’s multiple comparison test. Twenty substructures were assessed at each time point.

Article Snippet: , Gipc1 Polyclonal antibody , 14822-1-AP , Rabbit , 1:100 , Proteintech Group, Inc., Rosemont, IL, USA.

Techniques: Immunofluorescence, Staining, Marker, Comparison

Journal: Biomedicines

Article Title: Immunoexpression Patterns of Megalin, Cubilin, Caveolin-1, Gipc1 and Dab2IP in the Embryonic and Postnatal Development of the Kidneys in Yotari ( Dab1 −/− ) Mice

doi: 10.3390/biomedicines12071542

Figure Lengend Snippet: Immunofluorescence staining of embryonic wild-type and yotari mouse kidneys was performed using the Dab2IP ( a – d ) marker. Arrows indicate the immunoexpression patterns of Dab2IP in the metanephric mesenchyme (mm), glomeruli (g), renal vesicles (rv), ampullae (A), convoluted tubules (Ct) and collecting ducts (Cd), as shown on the 4′,6-diamidino-2-phenylindole (DAPI) nuclei staining image. Immunoexpression of Dab2IP, DAPI staining and the merged images of Gipc1 and DAPI was observed at embryonic days E13.5 and E15.5 in wild-type ( a , c ) and yotari ( b , d ) kidneys. Images were taken at ×40 magnification, and the scale bar represents 100 μm for all images. The percentages of Dab2IP ( e ) positive cells in the metanephric mesenchyme (mm), renal vesicles (rv) or glomeruli (g), convoluted tubules (Ct) and ampullae (A) or collecting ducts (Cd) of wild-type and yotari kidneys at embryonic days 13.5 and 15.5 are shown. Data are presented as the mean ± SD (vertical line) and analyzed using a two-way ANOVA followed by Tukey’s multiple comparison test. Significant differences are indicated by * p < 0.05, ** p < 0.01, **** p < 0.0001. Ten substructures were assessed at each time point.

Article Snippet: , Gipc1 Polyclonal antibody , 14822-1-AP , Rabbit , 1:100 , Proteintech Group, Inc., Rosemont, IL, USA.

Techniques: Immunofluorescence, Staining, Marker, Comparison

Journal: Molecular Pharmacology

Article Title: Modulation of ?-Opioid Receptor Signaling by RGS19 in SH-SY5Y Cells

doi: 10.1124/mol.112.081992

Figure Lengend Snippet: GIPC protein changes with RGS19. Whole-cell lysates were prepared from SH-SY5Y cells stably expressing either GFP shRNA or RGS19 shRNA as described under Materials and Methods. GIPC was detected as a single band at ∼37 KDa. This was decreased to ∼50% of control level in cells expressing shRNA against RGS19 compared with cells expressing shRNA against GFP (A). When SH-SY5Y cells were treated with (+) DAMGO (10 µM) overnight, GIPC was increased ∼50% compared with cells without DAMGO treatment (B). Quantified data from three sets of individual experiment are presented on the right side of the figure. Loading controls (α-tubulin) were not changed.

Article Snippet: Monoclonal anti-human GIPC antibody (clone AT1G10) was purchased from ATGen Co., Ltd. (Gyeonggi-do, South Korea).

Techniques: Stable Transfection, Expressing, shRNA, Control