TPP1,细胞生长抑制基因1蛋白抗体

产品编号HZ-8528R
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.
说 明 书0.1ml 0.2ml
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,
TPP1,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.
分 子 量40kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human TPP1/CLN2
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.
TPP1,细胞生长抑制基因1蛋白抗体PubMedPubMed
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.

TPP1,细胞生长抑制基因1蛋白抗体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.