anti-Retinoschisin 1 (RS1) Antikörper

RS1 encodes an extracellular protein that plays a crucial role in the cellular organization of the retina. Zusätzlich bieten wir Ihnen Retinoschisin 1 Kits (7) und Retinoschisin 1 Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
RS1 6247 O15537
Anti-Maus RS1 RS1 20147 Q9Z1L4
Anti-Ratte RS1 RS1 100125595  
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Top anti-Retinoschisin 1 Antikörper auf

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Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Human Kaninchen Unkonjugiert ICC, IHC, WB Western Blot; Sample:Porcine Eye lysate; Primary Ab: 1µg/ml Rabbit Anti-Human RS Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (Catalog: SAA544Rb19) Figure. Western Blot; Sample: Recombinant protein. 100 μg Anmelden zum Anzeigen 13 bis 16 Tage
Human Kaninchen Unkonjugiert ICC, IF, IHC (p) Immunocytochemistry/Immunofluorescence: RS1 Antibody  - Immunohistochemical staining of human duodenum shows cytoplasmic positivity in endocrine cells. Immunohistochemistry-Paraffin: RS1 Antibody  - Staining of retina shows moderate positivity in photoreceptor cells and nerve fibers. 100 μL Anmelden zum Anzeigen 10 bis 13 Tage
Human Maus Unkonjugiert WB Western Blot analysis of RS1 expression in transfected 293T cell line by RS1 MaxPab polyclonal antibody.Lane 1: RS1 transfected lysate(24.64 KDa).Lane 2: Non-transfected lysate. 50 μg Anmelden zum Anzeigen 11 bis 12 Tage
Human Kaninchen Unkonjugiert IF/ICC, IHC, IP, WB Western blot analysis of Pig Eye lysate, using Human RS Antibody (1 µg/ml) and HRP-conjugated Goat Anti-Rabbit antibody ( Western blot analysis of the recombinant protein. 100 μg Anmelden zum Anzeigen 71 Days
Human Kaninchen FITC ELISA   100 μg Anmelden zum Anzeigen 11 bis 16 Tage
Human Kaninchen Biotin ELISA   100 μg Anmelden zum Anzeigen 11 bis 16 Tage
Human Kaninchen HRP ELISA   100 μg Anmelden zum Anzeigen 11 bis 16 Tage
Human Kaninchen Unkonjugiert ELISA   100 μg Anmelden zum Anzeigen 11 bis 16 Tage
Human Kaninchen Unkonjugiert ELISA, IHC   100 μg Anmelden zum Anzeigen 11 bis 16 Tage
Human Maus Unkonjugiert IF/ICC, IHC, IP, WB   100 μg Anmelden zum Anzeigen 11 bis 18 Tage

Weitere Antikörper gegen Retinoschisin 1 Interaktionspartner

Human Retinoschisin 1 (RS1) Interaktionspartner

  1. molecular detail such as the precise localization of mutant protein in the cell as well as its ability to assemble into a functionally active oligomer might largely influence disease severity among XLRS patients

  2. These results establish that extracellular delivery of RS1 rescues the structural and functional deficits in the Rs1h knockout mouse model and that this ex vivo gene therapy approach can inhibit progression of disease.

  3. Taken together, RS1 mutation was found to segregate with retinoschisis phenotype while none of the other identified variations were co-segregating with the systemic defects. Hereby, we infer that the multisystemic defects harbored by the patient are a rare coexistence of XLRS, developmental delay, sensorineural hearing loss, and reduced axial tone reported for the first time in the literature.

  4. Results suggest a regulatory effect of retinoschisin on Na/K-ATPase signaling and localization, whereas Na/K-ATPase-dysregulation caused by retinoschisin deficiency could represent an initial step in XLRS pathogenesis.

  5. these findings support distinct mechanisms of pathology for two classes of X-linked retinoschisis -associated mutations in the retinoschisin assembly.

  6. A novel RS1 (97delT) mutation was identified in a Taiwanese family with X-linked retinoschisis (XLRS). This finding expands the RS1 mutation spectrum and may help to further understand the molecular pathogenesis of XLRS.

  7. Clinical and genetic characterization of affected homozygous females in XLRS affords the rare opportunity to explore the molecular mechanisms of XLRS and the manifestation of these mutations as disease in humans.

  8. A novel RS1 (304C > T) mutation in a Taiwanese family with X-linked retinoschisis.

  9. We identified a novel causative mutation of RS1 in a Chinese family with X-linked juvenile retinoschisis.

  10. the disease and p.Arg197Cys mutation of RS1 gene was identified

  11. X-linked retinoschisis despite striking differences in phenotypic presentation in affected subjects, homozygosity of one affected female, and seemingly dominant inheritance in three subsequent generations because of multiple consanguinity.

  12. Sequencing of the RS1 gene identified 16 mutations, nine of which were novel.

  13. Severe RS1 missense changes were associated with a lower ERG b/a ratio than were mild changes in X-linked retinoschisis suggesting the effect of the mutations on protein structure influenced the retinal dysfunction.

  14. Two novel exonic deletions within the RS1 gene locus, are reported.

  15. There is profound phenotypic variability in patients with XLRS. Nonsense, splice-site, or frame-shifting mutations in RS1 consistently caused electronegative bright-flash ERG, delayed flicker response, and abnormal PERG

  16. Four novel RS1 gene mutations have been described in male Polish patients with X-linked juvenile retinoschisis.

  17. aggregation propensity in the RS1 C110Y mutant is dependent upon the formation of suitable aggregating substrates for propagation of aggregation and not directly related to or determined by overall structural instability

  18. Clinical follow-up of an X-linked juvenile retinoschisis (XLRS) patient with a typical juvenile retinoschisis phenotype revealed no significant decline in visual acuity during this time period.

  19. Ten hemizygous mutations in RS1 were detected in patients from 14 of the 20 families with retinoschisis.

  20. Loss of RS1 due to mutations in the X-linked retinoschsis gene leads to splitting within the retinal layers.

Mouse (Murine) Retinoschisin 1 (RS1) Interaktionspartner

  1. Results suggest a regulatory effect of retinoschisin on Na/K-ATPase signaling and localization, whereas Na/K-ATPase-dysregulation caused by retinoschisin deficiency could represent an initial step in XLRS pathogenesis.

  2. retinoschisin is a novel regulator of MAP kinase signalling and exerts an anti-apoptotic effect on retinal cells.

  3. Based on this structure, we propose that RS1 couples neighboring membranes together through octamer-octamer contacts, perhaps modulated by interactions with other membrane components.

  4. Changes in Rs1-knockout mice were associated with age related alterations in photoreceptor morphology and transcription factor expression that suggest delayed photoreceptor maturation.

  5. Time line analysis after short-term treatment with dorzolamide failed to show short-, intermediate-, or long-term evidence of structural improvement in Rs1h(-/y) mice.

  6. The results of this study demonstrated that loss of Rs1 gene function has a significant impact on the expression of photoreceptor transcription factor network genes, and morphological and functional defects in young (P21) Rs1-KO mice.

  7. RS1 is needed for preservation of synaptic structures but not synaptogenesis in retinoschisis model.

  8. Data suggest that a CpG island enhancer and two CBRs may act in a combinatorial fashion to fine-tune RS1 transcript levels in the retina.

  9. Retinoschisin, the protein involved in the pathogenesis of X-linked juvenile retinoschisis, membrane association is severely impaired in the absence of ATP1A3 and ATP1B2.

  10. Upon Rs1 adsorption, phosphatidylserine and phosphatidylserine-containing mixed lipid bilayers underwent fast and extensive reorganization.

  11. Inactivation of Rs1h suggests a role of retinoschisin in retinal cell layer organization and synaptic structure

  12. After synthesis and secretion by the photoreceptors, retinoschisin [Xlrs1] reaches the surface of retinal cells and mediates interactions/adhesion between photoreceptor, bipolar, and Muller cells

  13. Mimics structural features of human X-linked juvenile retinoschisis. Knockout results in electronegative ERG waveform characteristic of human retinoschisis and implicates synaptic transmission deficit in the absence of retinoschisin protein.

  14. We have generated and characterized a mutant mouse line that was produced using ENU-based mutagenesis. The 44TNJ pedigree manifests with photoreceptor dysfunction and concurrent structural and functional aberrations at the post-receptoral level.

  15. analysis of two mature isoforms of retinoschisin in murine retina

  16. photoreceptor degeneration in the Rs1h(-/Y) mouse is due to apoptotic events peaking around postnatal day 18. Cell death is accompanied by increased expression of initiator and inflammatory caspases but not by downstream effector caspases.

  17. Retinoschisin protein(RS) is expressed in the pinealocytes but not in interstitial glial cells. The lack of structural abnormalities in the RS1(-/Y) mice suggests that RS serves a different function in the pineal gland than in the retina.

  18. These results demonstrate that RS1H has functional significance in the morphology and function of the synapse between photoreceptors and second-order neurons.

  19. confirmed the co-localization of retinoschisin with Na/K ATPase and SARM1 in photoreceptors and bipolar cells of retina tissue

  20. These results indicate that activated microglia from degenerating retinae express high levels of Dap12 and PU.1, and PU.1 controls the myeloid-specific regulation of Dap12 directly.

Retinoschisin 1 (RS1) Antigen-Profil

Protein Überblick

This gene encodes an extracellular protein that plays a crucial role in the cellular organization of the retina. The encoded protein is assembled and secreted from photoreceptors and bipolar cells as a homo-oligomeric protein complex. Mutations in this gene are responsible for X-linked retinoschisis, a common, early-onset macular degeneration in males that results in a splitting of the inner layers of the retina and severe loss in vision.

Genbezeichner und Symbole assoziert mit anti-Retinoschisin 1 (RS1) Antikörper

  • retinoschisin 1 (RS1) Antikörper
  • retinoschisin 1 S homeolog (rs1.S) Antikörper
  • retinoschisin 1 (rs1) Antikörper
  • retinoschisis (X-linked, juvenile) 1 (human) (Rs1) Antikörper
  • retinoschisin 1a (rs1a) Antikörper
  • retinoschisin 1 (Rs1) Antikörper
  • RS Antikörper
  • rs1 Antikörper
  • Rs1h Antikörper
  • tmgc1 Antikörper
  • Xlrs1 Antikörper
  • zgc:92703 Antikörper

Bezeichner auf Proteinebene für anti-Retinoschisin 1 (RS1) Antikörper

X-linked juvenile retinoschisis protein , retinoschisin , retinoschisis (X-linked, juvenile) 1 , retinoschisin 1 , retinoschisin-like , X-linked juvenile retinoschisis protein homolog , retinoschisis 1 homolog

6247 Homo sapiens
491762 Canis lupus familiaris
615193 Bos taurus
748866 Pan troglodytes
779332 Xenopus laevis
100124900 Xenopus (Silurana) tropicalis
100126075 Oryctolagus cuniculus
100127441 Sus scrofa
100423325 Macaca mulatta
20147 Mus musculus
445044 Danio rerio
100125595 Rattus norvegicus
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