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TPP1 Rabbit pAb (bs-8528R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-8528R
英文名称 TPP1 Rabbit pAb
中文名称 细胞生长抑制基因1蛋白抗体
别    名 CLN2; GIG1; LPIC; SCAR7; TPP-1; TPP1_HUMAN; TPP1; Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease (LPIC); Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I (TPP-I); 3.4.14.9;   
研究领域 细胞生物  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Rat (predicted: Human,Mouse,Rabbit,Pig,Sheep,Cow,Dog,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 40 kDa
检测分子量 61
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TPP1/CLN2: 401-500/563 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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 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, 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.

SWISS:
O14773

Gene ID:
1200

Database links:

Entrez Gene: 1200 Human

Omim: 607998 Human

SwissProt: O14773 Human

Unigene: 523454 Human



产品图片
25 ug total protein per lane of various lysates (see on figure) probed with TPP1 polyclonal antibody, unconjugated (bs-8528R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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