NBEAL2 Antibody, FITC conjugated (PACO46688)
- SKU:
- PACO46688
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | NBEAL2 Antibody, FITC conjugated (PACO46688) |
抗体コード: | PACO46688 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Neurobeachin-like protein 2 protein (1360-1574AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis. |
シノニム: | Neurobeachin-like protein 2, NBEAL2, KIAA0540 |
UniProt Protein Function: | NBEAL2: Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis. Defects in NBEAL2 are the cause of gray platelet syndrome (GPS). GPS is 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. Belongs to the WD repeat neurobeachin family. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Adaptor/scaffold Chromosomal Location of Human Ortholog: 3p21.31 Cellular Component: endoplasmic reticulum; extrinsic to membrane Molecular Function:phospholipid binding Biological Process: platelet formation Disease: Gray Platelet Syndrome |
NCBI Summary: | 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] |
UniProt Code: | Q6ZNJ1 |
NCBI GenInfo Identifier: | 149944548 |
NCBI Gene ID: | 23218 |
NCBI Accession: | NP_055990.1 |
UniProt Secondary Accession: | Q6ZNJ1,O60288, Q6P994, Q6UX91, Q8NAC9, |
UniProt Related Accession: | Q6ZNJ1 |
Molecular Weight: | 299,342 Da |
NCBI Full Name: | neurobeachin-like protein 2 |
NCBI Synonym Full Names: | neurobeachin like 2 |
NCBI Official Symbol: | NBEAL2 |
NCBI Official Synonym Symbols: | GPS; BDPLT4 |
NCBI Protein Information: | neurobeachin-like protein 2 |
UniProt Protein Name: | Neurobeachin-like protein 2 |
Protein Family: | Neurobeachin-like protein |
UniProt Gene Name: | NBEAL2 |
UniProt Entry Name: | NBEL2_HUMAN |