Product Name	TPP1
Chinese Name	细胞生长抑制基因1蛋白抗体
Alias	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.
Research Area	Cell biology  Neurobiology
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	40kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human TPP1/CLN2: 401-500/563
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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
Product Picture	Sample: Heart (Mouse) Lysate at 40 ug Primary: Anti- TPP1 (SL8528R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 40 kD Observed band size: 58 kD

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