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Rabbit Anti-NBEAL2/Gold Conjugated antibody (bs-19031R-Gold)
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-19031R-Gold
英文名称 Anti-NBEAL2/Gold
中文名称 胶体金标记的蛋白激酶锚定蛋白样蛋白2抗体
别    名 BDPLT4; GPS; NBEAL2; NBEL2_HUMAN; Neurobeachin-like protein 2; UNQ253/PRO290.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 细胞生物  发育生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Pig, Horse, Rabbit, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 303kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NBEAL2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 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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