CLN2/TPP1, Polyclonal Antibody

TPP1; tripeptidyl peptidase I; CLN2; GIG1; LPIC; MGC21297; ceroid-lipofuscinosis, neuronal 2, late infantile (Jansky-Bielschowsky disease); growth-inhibiting protein 1; lysosomal pepstatin insensitive protease; tripeptidyl aminopeptidase; tripeptidyl-pept; TPP1 antibody; tripeptidyl peptidase I antibody; CLN2 antibody; GIG1 antibody; LPIC antibody; MGC21297 antibody; ceroid-lipofuscinosis; neuronal 2; late infantile (Jansky-Bielschowsky disease) antibody; growth-inhibiting protein 1 antibody; lysosomal pepstatin insensitive protease antibody; tripeptidyl aminopeptidase antibody; tripeptidyl-peptidase I antibody; CLN2 / TPP1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-TPP1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Peptide ELISA (EIA), Western Blot (WB)

Peptide ELISA: Antibody detection limit dilution 1: 4000.
Western Blot: Approx 65kDa band observed in Human Placenta lysates (calculated MW of 61.2kDa according to NP_000382.3). Recommended concentration: 0.3-1ug/ml.

(0.3ug/ml) staining of Human Placenta lysate (35ug protein in RIPA buffer) with (B) and without (A) blocking with the immunising peptide. Primary incubation was 1 hour. Detected by chemiluminescence.

34,464 Da

tripeptidyl peptidase 1

tripeptidyl-peptidase 1

Tripeptidyl-peptidase 1

CLN2; TPP-1; LPIC; TPP-I  [Similar Products]

TPP1_HUMAN

This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008]

TPP1: 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. 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. Belongs to the peptidase S53 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.4.14.9; Secreted, signal peptide; Protease; Secreted; Mitochondrial

Chromosomal Location of Human Ortholog: 11p15

Cellular Component: lysosomal lumen; lysosome; melanosome; mitochondrion

Molecular Function: endopeptidase activity; metal ion binding; peptidase activity; peptide binding; protein binding; serine-type endopeptidase activity; serine-type peptidase activity; tripeptidyl-peptidase activity

Biological Process: bone resorption; central nervous system development; epithelial cell differentiation; lipid metabolic process; lysosome organization and biogenesis; nervous system development; neuromuscular process controlling balance; peptide catabolic process; protein catabolic process; proteolysis

Disease: Ceroid Lipofuscinosis, Neuronal, 2

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