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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.

Top Research Reagents

We have 2228 products for the study of Bernard-soulier Syndrome that can be applied to Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Coagulation Factor II/Thrombin Antibody
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
RNF130 Antibody
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

C4 binding protein A Antibody
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

ATP8A2 Antibody
NBP1-88899
Immunohistochemistry-Paraffin: ATP8A2 Antibody [NBP1-88899] - Staining of human testis shows strong cytoplasmic positivity in cells in seminiferus ducts.

Rabbit Polyclonal
Species Human
Applications IHC, IHC-P

ABCB6 Antibody
NBP1-91642
Immunocytochemistry/Immunofluorescence: ABCB6 Antibody [NBP1-91642] - Staining of human cell line U-251 MG shows localization to the Golgi apparatus.Immunohistochemistry-Paraffin: ABCB6 Antibody [NBP1-91642] - Staining of human testis shows cytoplasmic positivity.

Rabbit Polyclonal
Species Human
Applications ICC/IF, IHC, IHC-P

1 Publication
Von Willebrand Factor Antibody
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

14 Publications
Integrin alpha 2b/CD41 Antibody (745201) [Unconjugated]
MAB7616
Western blot shows lysates of human platelets. PVDF membrane was probed with 2 µg/mL of Mouse Anti-Human Integrin a2b/CD41 Monoclonal Antibody (Catalog # MAB7616) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (<a class=Human peripheral blood platelets were stained with Fluorescein-conjugated Anti-Mouse Integrin alpha 2b/CD41 (Clone P2) and either (A) Mouse IgG<SUB>1</SUB> Isotype Control (Catalog # <A class=NoLineLink href=

Mouse Monoclonal
Species Human
Applications WB, Flow, CyTOF-ready

6 Publications
CD42b/GPIb alpha Antibody [Unconjugated]
AF4067
Western blot shows lysates of human platelets. PVDF membrane was probed with 1 µg/mL of Sheep Anti-Human CD42b/GPIb a Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4067) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # <a class=<P align=left>Human whole blood CD41<SUP>+</SUP> platelets were stained with Sheep Anti-Human CD42b/GPIb  alpha Polyclonal Antibody (Catalog # AF4067) followed by NL637-conjugated anti sheep antibody (Catalog # NL011) and Human CD41 FITC-conjugated Monoclonal Antibody. Quadrant markers were set based on control antibody staining (Catalog # <A class=NoLineLink href=

Sheep Polyclonal
Species Human
Applications WB, Flow, CyTOF-ready

3 Publications
Thrombopoietin/THPO [Unconjugated]
288-TPN/CF
Measured in a cell proliferation assay using MO7e human megakaryocytic leukemic cells. The ED<sub>50</sub> for this effect is 5-15 ng/mL.


Species Human
Applications BA

12 Publications
PrPC Antibody
NB100-56605
Western Blot: PrPC Antibody [NB100-56605] - Analysis of BSE in (A) recombinant fusion protein containing amino acids 162-170 and (B) fusion partner without these amino acids, using this antibody at 5 ug/ml.

Rabbit Polyclonal
Species Avian, Bovine, Sheep
Applications WB

GP-IX Antibody (GRP-P)
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
Applications Flow, IP

Integrin beta 3/CD61 Antibody (SJ19-09)
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

6 Publications
non-muscle Myosin IIA Antibody (2B3)
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

2 Publications
CARD14 Antibody - Azide and BSA Free
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, IHC, IHC-P

Tenascin C Antibody (4C8MS) - BSA Free
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 (https://www.nature.com/doifinder/10.1038/ncomms11703), licensed under a CC-BY license.

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

     2 Reviews

26 Publications
Peroxiredoxin 2 Antibody (4E10-2D2)
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

Related Genes

Bernard-soulier Syndrome has been researched against:

Related Pathways

Bernard-soulier Syndrome has been linked to:

Related Diseases

Bernard-soulier Syndrome has been studied in relation to diseases such as:

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.