Recombinant Human HPS2 protein (ab127282)
Key features and details
- Expression system: Escherichia coli
- Purity: > 95% SDS-PAGE
- Tags: His-DHFR tag N-Terminus
- Suitable for: SDS-PAGE
製品の詳細
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製品名
Recombinant Human HPS2 protein -
精製度
> 95 % SDS-PAGE.
Purified via His tag -
発現系
Escherichia coli -
アクセッション番号
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タンパク質長
Protein fragment -
Animal free
No -
由来
Recombinant -
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生物種
Human -
予測される分子量
16 kDa -
領域
944 to 1094 -
タグ
His-DHFR tag N-Terminus
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関連製品
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Related Products
特性
Our Abpromise guarantee covers the use of ab127282 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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アプリケーション
SDS-PAGE
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製品の状態
Lyophilized -
Concentration information loading...
前処理および保存
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保存方法および安定性
Shipped at 4°C. Store at -20ºC.
Constituents: 0.32% Tris HCl, 0.58% Sodium chloride
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再構成Reconstitute with water to desired concentration.
関連情報
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別名
- Adapter related protein complex 3 beta 1 subunit
- Adapter-related protein complex 3 subunit beta-1
- Adaptor protein complex AP-3 subunit beta-1
see all -
機能
Subunit of non-clathrin- and clathrin-associated adaptor protein complex 3 that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. AP-3 appears to be involved in the sorting of a subset of transmembrane proteins targeted to lysosomes and lysosome-related organelles. -
組織特異性
Ubiquitously expressed. -
関連疾患
Defects in AP3B1 are the cause of Hermansky-Pudlak syndrome type 2 (HPS2) [MIM:608233]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. HPS2 differs from the other forms of HPS in that it includes immunodeficiency in its phenotype and patients with HPS2 have an increased susceptibility to infections. -
配列類似性
Belongs to the adaptor complexes large subunit family. -
翻訳後修飾
Phosphorylated on serine residues. -
細胞内局在
Golgi apparatus. Cytoplasmic vesicle > clathrin-coated vesicle membrane. Golgi apparatus. Component of the coat surrounding the cytoplasmic face of coated vesicles located at the Golgi complex. - Information by UniProt
プロトコール
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
データシートおよび資料
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Datasheet download
参考文献 (0)
ab127282 は論文での使用が確認できていません。