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货号： bs-19103R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19103R-RBITC
- 英文名称
- Anti-Munc 13-4/RBITC
- 中文名称
- 罗丹明（RBITC）标记的UNC13D蛋白抗体
- 别 名
- FHL 3; FHL3; FLJ00067; HLH 3; HLH3; HPLH 3; HPLH3; Jinx; Munc13 4; Munc13-4; Protein unc 13 homolog D; Protein unc-13 homolog D; UN13D_HUMAN; Unc 13 homolog D; UNC 13D; Unc-13 homolog D (C. elegans); Unc13 homolog D (C elegans); Unc13 homolog D; UNC13, C. elegans, homolog of, D; UNC13D; Unc13h4
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 t-淋巴细胞
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rhesus monkey
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 123kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Munc 13-
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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 antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
This gene encodes a protein that is a member of the UNC13 family, containing similar domain structure as other family members but lacking an N-terminal phorbol ester-binding C1 domain present in other Munc13 proteins. The protein appears to play a role in vesicle maturation during exocytosis and is involved in regulation of cytolytic granules secretion. Mutations in this gene are associated with familial hemophagocytic lymphohistiocytosis type 3, a genetically heterogeneous, rare autosomal recessive disorder. [provided by RefSeq, Jul 2008]
Function:
Plays a role in cytotoxic granule exocytosis in lymphocytes. Required for both granule maturation and granule docking and priming at the immunologic synapse. Regulates assembly of recycling and late endosomal structures, leading to the formation of an endosomal exocytic compartment that fuses with perforin-containing granules at the immunologic synapse and licences them for exocytosis. Regulates Ca(2+)-dependent secretory lysosome exocytosis in mast cells.
Subcellular Location:
Cytoplasm. Membrane. Late endosome. Recycling endosome. Lysosome. Colocalizes with cytotoxic granules at the plasma membrane. Localizes to endosomal exocytic vesicles.
Tissue Specificity:
Expressed at high levels in spleen, thymus and leukocytes. Also expressed in lung and placenta, and at very low levels in brain, heart, skeletal muscle and kidney. Expressed in cytotoxic T-lymphocytes (CTL) and mast cells.
DISEASE:
Defects in UNC13D are the cause of hemophagocytic lymphohistiocytosis familial type 3 (FHL3) [MIM:608898]; also known as HPLH3. Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous, rare autosomal recessive disorder. It is characterized by immune dysregulation with hypercytokinemia and defective natural killer cell function. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits, and ataxia. Hemophagocytosis is a prominent feature of the disease, and a non-malignant infiltration of macrophages and activated T lymphocytes in lymph nodes, spleen, and other organs is also found.
Similarity:
Belongs to the unc-13 family.
Contains 2 C2 domains.
Contains 1 MHD1 (MUNC13 homology domain 1) domain.
Contains 1 MHD2 (MUNC13 homology domain 2) domain.
Database links:Entrez Gene: 201294Human
Entrez Gene: 70450Mouse
Entrez Gene: 192177Rat
Entrez Gene: 704431 Rhesus monkey
Omim: 608897Human
SwissProt: Q70J99Human
SwissProt: B2RUP2Mouse
SwissProt: Q9R189Rat
Unigene: 41045Human
Unigene: 259460Mouse
Unigene: 198919Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.