TPP1细胞生长抑制基因1蛋白抗体

TPP1细胞生长抑制基因1蛋白抗体

价格: ¥1380 - 2200

品牌:LMAI Bio

货号:LM-8528R

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供应商 :上海联迈生物工程有限公司

库存 :大量

目录编号 :LM-8528R

克隆性 :多克隆

抗原来源 :Rabbit

保质期 :1年

抗体英文名 :TPP1

抗体名 :细胞生长抑制基因1蛋白抗体

宿主 :Rabbit

适应物种 :Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,

免疫原 :KLH conjugated synthetic peptide derived from human TPP1/CLN2:401-500/563

亚型 :IgG

形态 :Lyophilized or Liquid

应用范围 :WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:50-200 (石蜡切片需做抗原修复)

浓度 :1mg/ml

保存条件 :Store at -20 °C

规格 :100ul  200ul

TPP1细胞生长抑制基因1蛋白抗体
英文名称 TPP1
中文名称 细胞生长抑制基因1蛋白抗体
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 lipofuscinosis neuronal 2 late infantile antibody CLN 2; CLN2; CLN-2; GIG 1; GIG1; Growth inhibiting protein 1; LPIC; Lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; TPP 1; TPP I; TPP-1; TPP-I; Tpp1; TPP1_HUMAN; TPPI; Tripeptidyl aminopeptidase; Tripeptidyl peptidase I; Tripeptidyl-peptidase 1; Tripeptidyl-peptidase I.  
规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
说 明 书 100ul  200ul
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, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.
分 子 量 40kDa
细胞定位 细胞浆 
性    状 Lyophilized or 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
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.
PubMed PubMed
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 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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

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