Rabbit Anti-NBEAL2 antibody

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BDPLT4; GPS; NBEAL2; NBEL2_HUMAN; Neurobeachin-like protein 2; UNQ253/PRO290.

Cat:

SL19031R

Species Reactivity：

(predicted: Human,Mouse,Rat,Pig,Horse,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human NBEAL2:101-200/2754

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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.

SWISS:
Q6ZNJ1

Gene ID:
23218

Database links:

Entrez Gene: 23218 Human

Omim: 614169 Human

SwissProt: Q6ZNJ1 Human

Unigene: 437043 Human

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