Home > Product > Antibody > Rabbit Anti-NBEAL2/FITC Conjugated antibody
BDPLT4; GPS; NBEAL2; NBEL2_HUMAN; Neurobeachin-like protein 2; UNQ253/PRO290.
Cat:
SL19031R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Pig,Horse,Rabbit,)
Immunogen:
KLH conjugated synthetic peptide derived from human NBEAL2
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
303kDa
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Unit:
Price: $
Product PDFs
Datasheet:


background:
The protein encoded by this gene contains a beige and Chediak-Higashi (BEACH) domain and multiple WD40 domains, and may play a role in megakaryocyte alpha-granule biogenesis. Mutations in this gene are a cause of gray platelet syndrome. [provided by RefSeq, Dec 2011]

Function:
Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis.

Subcellular Location:
Endoplasmic reticulum.

Tissue Specificity:
Expressed in megakaryocytes.

DISEASE:
Gray platelet syndrome (GPS) [MIM:139090]: A rare platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with mild to moderate bleeding tendency and moderate thrombocytopenia. The platelets are enlarged and have a gray appearance on light microscopy of Wright-stained peripheral blood smears due to decreased granules.

Similarity:
Belongs to the WD repeat neurobeachin family.
Contains 1 BEACH domain.
Contains 5 WD repeats.

Database links:

Entrez Gene: 23218 Human

Omim: 614169 Human

SwissProt: Q6ZNJ1 Human

Unigene: 437043 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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