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货号： bsm-51262M-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bsm-51262M-Cy3
- 英文名称
- Anti-PPT1/Cy3
- 中文名称
- Cy3标记的小鼠抗棕榈酰蛋白硫酯酶1单克隆抗体
- 别 名
- CLN1; INCL; Palmitoyl protein hydrolase 1; Palmitoyl protein thioesterase 1; Palmitoyl-protein hydrolase 1; Palmitoyl-protein thioesterase 1; PPT; PPT-1; PPT1; PPT1_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 信号转导
- 抗体来源
- Mouse
- 克隆类型
- Monoclonal
- 克 隆 号
- 10G4
- 交叉反应
- 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
- 31kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PPT1
- 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:
The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008]
Function:
Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons (PubMed:8816748)
Subcellular Location:
Lysosome.
DISEASE:
Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1) [MIM:256730]. A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late-infantile, juvenile, and adult onset have been reported. 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 lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD).
Similarity:
Belongs to the palmitoyl-protein thioesterase family.
Database links:Entrez Gene: 281421 Cow
Entrez Gene: 5538 Human
Entrez Gene: 19063 Mouse
Entrez Gene: 29411 Rat
Omim: 600722 Human
SwissProt: P45478 Cow
SwissProt: P50897 Human
SwissProt: O88531 Mouse
SwissProt: P45479 Rat
Unigene: 3873 Human
Unigene: 277719 Mouse
Unigene: 1574 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.