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Bernard-soulier Syndrome: Disease Bioinformatics

Bernard-Soulier Syndrome, also known as hemorrhagiparous thrombocytic dystrophy, is an extremely rare genetic disorder that is characterized by the absence of glycoprotein which is vital to clot formation. The symptoms of Bernard-Soulier Syndrome include postoperative bleeding, easy bruising, and bleeding gums. Bernard-Soulier Syndrome is usually treated with medications that promote clotting, but can also be treated with a platelet transfusion. There is no cure for Bernard-Soulier Syndrome, but symptoms sometimes subside as the person gets older.

Bernard-soulier Syndrome Bioinformatics Tool

Laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on co-citations. Explore more on Bernard-soulier Syndrome below! For more information on how to use Laverne, please read the How to Guide.
Vizit™, under license from BioVista Inc.

Top Research Reagents

We have 1359 products for the study of Bernard-soulier Syndrome that can be applied to Western Blot, Flow Cytometry, Immunocytochemistry/Immunofluorescence, Immunohistochemistry from our catalog of antibodies and ELISA kits.

NB600-586
Dual RNAscope ISH-IHC: Von Willebrand Factor Antibody [NB600-586] - Formalin-fixed paraffin-embedded tissue sections of human metastatic tonsil were probed for vWF mRNA (ACD RNAScope probe, catalog # 5860461; Fast Red chromogen, ACD catalog # 322500). Adjacent tissue section was processed for immunohistochemistry using rabbit polyclonal  (Novus catalog # NB600-586) at 1:500 dilution for 1 hour at room temperature followed by incubation with the anti-rabbit IgG VisUCyte HRP Polymer Antibody (Catalog # VC003) and DAB chromogen (yellow-brown). Tissue was counterstained with hematoxylin (blue).Immunohistochemistry: Von Willebrand Factor Antibody [NB600-586] - Formalin fixed paraffin embedded human tonsil stained with Factor VIII antibody.

Rabbit Polyclonal
Species Human, Mouse, Canine
Applications ICC/IF, IHC, IHC-Fr

     3 Reviews

4 Publications
NBP1-81858
Western Blot: RNF130 Antibody [NBP1-81858] - Lane 1: Marker  [kDa] 230, 130, 95, 72, 56, 36, 28, 17, 11.  Lane 2: Human cell line RT-478Immunohistochemistry-Paraffin: RNF130 Antibody [NBP1-81858] - Staining of human kidney shows strong cytoplasmic positivity in cells in tubules.

Rabbit Polyclonal
Species Human
Applications WB, IHC, IHC-P

NB110-68136
Flow (Intracellular): Tenascin C Antibody (4C8MS) [NB110-68136] - Figure A: Intracellular stain performed on U87MG Cells with Tenascin C (4C8MS) antibody NB110-68136 (blue) and a matched isotype control NBP1-97005 (orange). Cells were fixed with 4% paraformaldehyde, following fixation, cells were permeabilized with 0.1% saponin. Cells were incubated in an antibody dilution of 1 ug/mL for 30 minutes at room temperature, followed by mouse F(ab)2 IgG (H+L) APC-conjugated secondary antibody [F0101B, R&D Systems].Figure B: U87MG Cells were either untreated (orange) or treated with 3uM Monensin (blue). An intracellular stain was performed with Tenascin C (4C8MS) antibody NB110-68136. Cells were fixed with 4% paraformaldehyde, following fixation, cells were permeabilized with 0.1% saponin. Cells were incubated in an antibody dilution of 1 ug/mL for 30 minutes at room temperature, followed by mouse F(ab)2 IgG (H+L) APC-conjugated secondary antibody [F0101B, R&D Systems].Western Blot: Tenascin C Antibody (4C8MS) [NB110-68136] - Regulation of Tenascin C expression and its effect on fibrotic responses. Confluent foreskin fibroblasts were incubated with TGF-beta (10 ng ml-1 or indicated concentrations) or Tenascin C (TNC) for 24 or 72 h or indicated periods. Whole-cell lysates, culture media and RNA were examined by western analysis (upper panels) and qPCR (lower panel). Representative immunoblots or qPCR results (means+/-s.e.m. of triplicate determinations). S, secreted; L, lysates. Image collected and cropped by CiteAb from the following publication (http://www.nature.com/doifinder/10.1038/ncomms11703), licensed under a CC-BY licence.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, ELISA, Flow

     2 Reviews

22 Publications
NB100-65904
Flow Cytometry: GP-IX Antibody (GRP-P) [NB100-65904] - Analysis using the PE conjugate of NB100-65904. Staining of human peripheral blood platelets.Flow Cytometry: GP-IX Antibody (GRP-P) [NB100-65904] - Staining of human peripheral blood platelets with Mouse anti Human GP-IX

Mouse Monoclonal
Species Human, Canine, Mustelid
Applications Flow, IP

NBP2-55197
Immunocytochemistry/Immunofluorescence: ATP8A2 Antibody [NBP2-55197] - Staining of human cell line PC-3 shows localization to plasma membrane.

Rabbit Polyclonal
Species Human
Applications ICC/IF

NBP1-28457
Immunohistochemistry-Paraffin: CD42b/GPIb alpha Antibody (MM2/174) [NBP1-28457] - Analysis on HCL-decalcified, paraffin embedded human bone marrow core labeling for CD42b (clone: MM2/174) at 1/100. IHC-P image submitted by a verified customer review. Flow Cytometry: CD42b/GPIb alpha Antibody (MM2/174) [NBP1-28457] - Human peripheral blood platelets were stained with Mouse Anti-Human CD42b-PE NBP1-28503.

Mouse Monoclonal
Species Human
Applications Flow, IHC, IHC-P

     1 Review

NBP1-84579
Immunohistochemistry-Paraffin: Integrin alpha 2b/CD41 Antibody [NBP1-84579] - Staining in human bone marrow and stomach tissues using anti-ITGA2B antibody. Corresponding ITGA2B RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Integrin alpha 2b/CD41 Antibody [NBP1-84579] - Staining of human bone marrow, colon, liver and lymph node using Anti-ITGA2B antibody NBP1-84579 (A) shows similar protein distribution across tissues to independent antibody NBP1-84582 (B).

Rabbit Polyclonal
Species Human
Applications IHC, IHC-P

H00004627-M03
Western Blot: non-muscle Myosin IIA Antibody (2B3) [H00004627-M03] - Analysis of MYH9 expression in transfected 293T cell line by MYH9 monoclonal antibody (M03), clone 2B3. Lane 1: MYH9 transfected lysate (Predicted MW: 10.01 KDa). Lane 2: Non-transfected lysate.Immunocytochemistry/Immunofluorescence: non-muscle Myosin IIA Antibody (2B3) [H00004627-M03] - Bovine ovarian granulosa cells. Anti Myosin IIA immunofluorescent staining (green). DNA in counterstained with DAPI (blue). Control was obtained by omitting the primary antibody. Fixation: 4% PF for 7 minutes at rt. Permeabilization: 0.1% Triton-X in PBS for 7 minutes. Blocking: 20% donkey serum, 1% BSA in PBS, 1 hour at rt. Primary antibody: 1:50 or 1:100 in 1% BSA/PBS over night at 4C. Secondary antibody: donkey anti mouse -Alexa 488 (Invitrogen), 1:500. DNA Counterstaining: mounting medium with DAPI. Negative CTRL was performed by omitting the primary antibody, images were captured under an epifluorescence microscope. Image from verified customer review.

Mouse Monoclonal
Species Human, Bovine
Applications WB, ELISA, ICC/IF

     1 Review

NBP2-67416
Western Blot: Integrin beta 3/CD61 Antibody (SJ19-09) [NBP2-67416] - Analysis of Integrin beta 3 on different lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature. Positive control: Lane 1: HUVEC cell lysateLane 2: mouse marrow tissue lysateImmunocytochemistry/Immunofluorescence: Integrin beta 3/CD61 Antibody (SJ19-09) [NBP2-67416] - Staining Integrin beta 3 in HepG2 cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilized with 0.25% Triton X-100/PBS.

Rabbit Monoclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

     2 Reviews

1 Publication
NBP1-58268
Western Blot: Coagulation Factor II/Thrombin Antibody [NBP1-58268] - 721_B tissue lysate at a concentration of 1ug/ml.Immunohistochemistry: Coagulation Factor II/Thrombin Antibody [NBP1-58268] - Analysis of human liver after heat-induced Antigen retrieval. Antibody concentration 5 ug/ml.

Rabbit Polyclonal
Species Human, Mouse
Applications WB, IHC, IHC-P

     1 Review

3 Publications
H00007001-M01
Western Blot: Peroxiredoxin 2 Antibody (4E10-2D2) [H00007001-M01] - PRDX2 monoclonal antibody (M01), clone 4E10-2D2 Analysis of PRDX2 expression in Hela.Immunocytochemistry/Immunofluorescence: Peroxiredoxin 2 Antibody (4E10-2D2) [H00007001-M01] - Analysis of monoclonal antibody to PRDX2 on HeLa cell. Antibody concentration 10 ug/ml.

Mouse Monoclonal
Species Human
Applications WB, ELISA, ICC/IF

     1 Review

6 Publications
NBP2-67569
Western Blot: PrPC Antibody (SC57-05) [NBP2-67569] - Western blot analysis of PrPC protein on rat brain tissue lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (1/500) was used in 5% BSA at room temperature for 2 hoursImmunocytochemistry/Immunofluorescence: PrPC Antibody (SC57-05) [NBP2-67569] - Staining PrPC in N2A cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Rabbit Monoclonal
Species Human, Mouse, Rat
Applications WB, Flow, ICC/IF

NBP1-88262
Immunohistochemistry-Paraffin: C4 binding protein A Antibody [NBP1-88262] - Staining of human colon, kidney, liver and lung using Anti-C4BPA antibody NBP1-88262 (A) shows similar protein distribution across tissues to independent antibody NBP1-88263 (B).Western Blot: C4 binding protein A Antibody [NBP1-88262] - Analysis in control (vector only transfected HEK293T lysate) and C4BPA over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (3.1 kDa) in mammalian HEK293T cells).

Rabbit Polyclonal
Species Human
Applications WB, IHC, IHC-P

NBP2-58327
Western Blot: ABCB6 Antibody [NBP2-58327] - Analysis in human cell line RT-4 and human cell line U-251 MG.Immunocytochemistry/Immunofluorescence: ABCB6 Antibody [NBP2-58327] - Staining of human cell line U-251 MG shows localization to nucleoplasm & the Golgi apparatus. Antibody staining is shown in green.

Rabbit Polyclonal
Species Human, Mouse
Applications WB, ICC/IF

1 Publication
NBP2-92873
Western Blot: CARD14 Antibody [NBP2-92873] - Analysis of extracts of various cell lines, using CARD14 at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 10s.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

288-TPN/CF


Species Human

6 Publications

Related Genes

Bernard-soulier Syndrome has been researched against:

Related PTMs

Bernard-soulier Syndrome has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Bernard-soulier Syndrome is also known as bernard-soulier syndrome, macrothrombocytopenia, familial bernard-soulier type, von willebrand factor receptor deficiency, hemorrhagiparous thrombocytic dystrophy, hemorrhagic dystrophic thrombocytopenia, platelet glycoprotein 1b, deficiency of, deficiency of platelet glycoprotein 1b, giant platelet syndrome (disorder), bernard - soulier thrombopathy, thrombopathy, bernard-soulier, bernard soulier syndrome, giant platelet disorder, giant platelet syndrome, giant platelet disease, thrombopathy, hemorrhage, gigantism, bss.