Polyclonal CLN2; Anti-CLN2; TPP1; CLN-2; tripeptidyl aminopeptidase; LPIC; CLN2; lysosomal pepstatin insensitive protease; CLN 2; growth-inhibiting protein 1; MGC21297

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

Polyclonal

IgG

Rabbit

CLN2 antibody was purified by antigen-affinity chromatography

Supplied as a concentrated soloution containing 0.1M Tris, 0.1M Glycine, 10% Glycerol (pH 7.0). 0.01% Thimerosal was added as a preservative.

1 mg/ml (lot specific)

Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Small volumes of anti-CLN2 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.

Affinity purified Rabbit polyclonal CLN2 antibody

Proteases, Inhibitors, & Enzymes; Purified Polyclonal Antibodies

Western Blot (WB)

WB: 1:500-1:3000

Western blot analysis of 30 ug of whole cell lysate (A: A431) using a 7.5 % SDS PAGE gel and CLN2 antibody at a dilution of 1:1000

61 kDa

tripeptidyl peptidase I

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

Chromosomal Location of Human Ortholog: 11p15

Cellular Component: lysosomal lumen; mitochondrion; lysosome; melanosome

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

Biological Process: nervous system development; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; epithelial cell differentiation; lysosome organization and biogenesis; unfolded protein response; peptide catabolic process; protein catabolic process; lipid metabolic process; neuromuscular process controlling balance; proteolysis; bone resorption

Disease: Ceroid Lipofuscinosis, Neuronal, 2

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