细胞生长抑制基因1蛋白抗体
规格：1mg/1ml
英文名： TPP1
别名： Cell growth inhibiting gene 1 protein; Cell growth-inhibiting gene 1 protein; Ceroid lipofuscinosis neuronal 2; Ceroid lipofuscinosis neuronal 2 late infantile (Jansky Bielschowsky disease); Ceroid li
分子量： 40kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human TPP1/CLN
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆
细胞生长抑制基因1蛋白抗体产品介绍：background: Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. Function: Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus (By similarity). Subunit: Monomer. Subcellular Location: Lysosome. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV. Tissue Specificity: Detected in all tissues examined with highest levels in heart and placenta细胞生长抑制基因1蛋白抗体 and relatively similar levels in other tissues. Post-translational modifications: Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity. DISEASE: Involvement in disease: Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2) . 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, 细胞生长抑制基因1蛋白抗体dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles. Similarity: Belongs to the peptidase S53 family. Database links: UniProtKB/Swiss-Prot: O14773.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
细胞生长抑制基因1蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid