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货号： bs-18731R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-18731R-AF555
- 英文名称
- Anti-mucolipin 1/AF555
- 中文名称
- AF555标记的粘脂蛋白1抗体
- 别 名
- MCLN1_HUMAN ; Mcoln 1 ; Mcoln1 ; MG 2 ; MG-2 ; MG2 ; ML 4 ; ML4 ; MLIV ; MST080 ; MSTP080 ; Mucolipidin ; Mucolipin-1 ; Mucolipin1 ; TRP ML1 ; TRPML1.
- 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 信号转导 跨膜蛋白
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, Monkey, Cat,
- 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
- 65kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human mucolipin 1
- 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 memberof the transient receptor potential (TRP) cation channel gene family. The transmembrane protein localizes to intracellular vesicular membranes including lysosomes, and functions in the late endocytic pathway and in the regulation of lysosomal exocytosis. The channel is permeable to Ca(2+), Fe(2+), Na(+), K(+), and H(+), and is modulated by changes in Ca(2+) concentration. Mutations in this gene result in mucolipidosis type IV. [provided by RefSeq, Oct 2009]
Function:
Cation channel probably playing a role in the endocytic pathway and in the control of membrane trafficking of proteins and lipids. Could play a major role in Ca(2+) transport regulating lysosomal exocytosis.
Subcellular Location:
Cell membrane. Late endosome membrane. Lysosome membrane.Entrez Gene: 57192 HumanEntrez Gene: 94178 MouseEntrez Gene: 288371 RatOmim: 605248 HumanSwissProt: Q9GZU1 HumanSwissProt: Q99J21 MouseUnigene: 567548 HumanUnigene: 631858 HumanUnigene: 8356 Mouse
Tissue Specificity:
Widely expressed in adult and fetal tissues.
DISEASE:
Defects in MCOLN1 are the cause of mucolipidosis type IV (MLIV) [MIM:252650]; also known as sialolipidosis. MLIV is an autosomal recessive lysosomal storage disorder characterized by severe psychomotor retardation and ophthalmologic abnormalities, including corneal opacity, retinal degeneration and strabismus. Storage bodies of lipids and water-soluble substances are seen by electron microscopy in almost every cell type of the patients. Most patients are unable to speak or walk independently and reach a maximal developmental level of 1-2 years. All patients have constitutive achlorhydia associated with a secondary elevation of serum gastrin levels. MLIV may be due to a defect in sorting and/or transport along the late endocytic pathway. MLIV is found at relatively high frequency among Ashkenazi Jews.
Similarity:
Belongs to the transient receptor (TC 1.A.4) family. Polycystin subfamily. MCOLN1 sub-subfamily.
Database links:Entrez Gene: 57192Human
Entrez Gene: 94178Mouse
Entrez Gene: 288371Rat
Omim: 605248Human
SwissProt: Q9GZU1Human
SwissProt: Q99J21Mouse
Unigene: 567548Human
Unigene: 631858Human
Unigene: 8356Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.