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货号： bs-8016R-AP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8016R-AP
- 英文名称
- Anti-CLN3/AP
- 中文名称
- 碱性磷酸酶（AP）标记的神经细胞蜡样质脂褐质沉积病蛋白CLN3抗体
- 别 名
- Batten disease protein; Battenin; BTS; Ceroid lipofuscinosis neuronal 3; Ceroid lipofuscinosis neuronal 3 juvenile (Batten Spielmeyer Vogt disease); Ceroid lipofuscinosis neuronal 3 juvenile; CLN 3; MGC102840; Protein CLN3; CLN3_HUMAN.
- 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 神经生物学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Cow, Horse, Rabbit, Monkey, macaque
- 产品应用
- WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 48kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human CLN3
- 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 involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene.
Function:
Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.
Subunit:
Interacts with DCTN1 and KIF3A. Interacts with RAB7A and RILP.
Subcellular Location:
Lysosome membrane; Multi-pass membrane protein. Late endosome
Post-translational modifications:
Highly glycosylated.
Farnesylation is important for trafficking to lysosomes.
DISEASE:
Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3.
Similarity:
Belongs to the battenin family.
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.