Mouse anti-Human TPP-1(Tripeptidyl-peptidase 1, TPP-l, Tripeptidyl aminopeptidase, Lysosomal pepstatin-insensitive protease, LPIC, Cell growth-inhibiting gene 1 protein, TPP1, CLN2) Monoclonal Antibody | anti-TPP1 antibody

TPP-1(Tripeptidyl-peptidase 1, TPP-l, Tripeptidyl aminopeptidase, Lysosomal pepstatin-insensitive protease, LPIC, Cell growth-inhibiting gene 1 protein, TPP1, CLN2)

Cat. Num. AAA604509

• Gene Names: TPP1; CLN2; GIG1; LPIC; TPP-1; MGC21297
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity Purified; Purified by affinity chromatography.
• Synonyms: TPP-1(Tripeptidyl-peptidase 1; TPP-l; Tripeptidyl aminopeptidase; Lysosomal pepstatin-insensitive protease; LPIC; Cell growth-inhibiting gene 1 protein; TPP1; CLN2); Monoclonal Antibody; Anti -TPP-1(Tripeptidyl-peptidase 1; anti-TPP1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG
• Clone Number: 9G454
• Specificity: Recognizes human TPP1(195-305).
• Purity/Purification: Affinity Purified; Purified by affinity chromatography.
• Form/Format: Supplied as a lyophilized powder in PBS, pH 7.6. Reconstitute with 100ul sterile ddH2O.

• Applicable Applications for anti-TPP1 antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in ELISA, Western Blot and Immunohistochemistry.
• Immunogen: Partial recombinant protein of human TPP1 aa195-305 with a GST tag.
• Preparation and Storage: Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile 40-50% glycerol, ddH2O. Reconstituted product is stable for 12 months at -20 degree C. Aliquot and store at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Immunohistochemistry (IHC)

(Immunohistochemical detection of TPP1 in formalin fixed, paraffin-embedded human slivary gland using mouse monoclonal antibody to human TPP1.)

Western Blot (WB)

(Western blot detection of TPP1 in human epidermoid carcinoma lysate when using monoclonal antibody to human TPP1.)

Related Product Information for anti-TPP1 antibody

TPP1 is a lysosomal serine protease with tripeptidyl-peptidase I activity. It may act as a non-specific lysosomal peptidase which releases an N-terminal tripeptide from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. TPP1 is detected in lysosomes and melanosomes in all tissues but it is highest in the heart and placenta. TPP1 is activated by autocatalytic proteolytic processing upon acidification within lysosomes. Defects in TPP1 cause classical late-infantile neuronal ceroid lipofuscinosis (LINCL) which is also known as ceroid lipofuscinosis 2 (CLN2). It is a fatal childhood neurodegenerative disease characterised by progressive visual and mental deterioration, motor disturbance, epilepsy and behavioral changes.

Product Categories/Family for anti-TPP1 antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 5729770
• NCBI GeneID: 1200
• NCBI Accession #: NP_000382.3
• NCBI GenBank Nucleotide #: NM_000391.3
• UniProt Accession #: O14773; Q53HT1; Q5JAK6; Q6UX56; Q71JP6; Q96C37; Q53HP2
• Molecular Weight: 61,248 Da
• NCBI Official Full Name: tripeptidyl-peptidase 1 preproprotein
• NCBI Official Synonym Full Names: tripeptidyl peptidase I
• NCBI Official Symbol: TPP1
• NCBI Official Synonym Symbols: CLN2; GIG1; LPIC; TPP-1; MGC21297
• NCBI Protein Information: tripeptidyl-peptidase 1; OTTHUMP00000164588; tripeptidyl aminopeptidase; growth-inhibiting protein 1; cell growth-inhibiting gene 1 protein; lysosomal pepstatin insensitive protease
• UniProt Protein Name: Tripeptidyl-peptidase 1
• Protein Family: Tripeptidyl-peptidase
• UniProt Gene Name: TPP1
• UniProt Synonym Gene Names: CLN2
• UniProt Entry Name: TPP1_HUMAN

NCBI Description

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]

Uniprot Description

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: Mitochondrial; Protease; EC 3.4.14.9; 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