KLH conjugated synthetic peptide derived from human TPP1/CLN2:401-500/563
Format：
Liquid
Storage instructions：
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration：
1mg/ml
Clonality：
Polyclonal
Isotype：
IgG
Applications：
WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500IF=1:50-200（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host：
Rabbit
Product Overview：
Sample: Heart (Mouse) Lysate at 40 ugPrimary: Anti- TPP1 (SL8528R) at 1/1000 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 40 kDObserved band size: 58 kD
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.
