TPP1; CLN2; GIG1; LPIC; TPP-1

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

Chromosome: 11; NC_000011.10 (6612766..6619461, complement). Location: 11p15

Monoclonal

IgG2

(9V1)

Mouse

Lyophilized

Lyophilized samples are stable for 2 years from date of receipt when stored at -70 degree C. Reconstituted antibody can be aliquoted and stored frozen at < -20 degree C for at least for six months without detectable loss of activity.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-CLN-2 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.

Tripeptidyl-peptidase I (TPP1), also known as CLN2, is a lysosomal serine protease detected in all tissues examined, with highest levels shown in heart and placenta. It is an aminopeptidase that cleaves tripeptides from the free N-terminus of polypeptides and may also have endopeptidase activity. Defects in TPP1 are the cause of classical late-infantile neuronal ceroid lipofusionosis, also known as ceroid lipofusionosis neuronal 2 (CLN2), a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy, and behavioral changes.

Western Blot (WB)

61,248 Da

tripeptidyl peptidase I

tripeptidyl-peptidase 1; tripeptidyl aminopeptidase; growth-inhibiting protein 1; cell growth-inhibiting gene 1 protein; lysosomal pepstatin insensitive protease

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]

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.

Catalytic activity: Release of an N-terminal tripeptide from a polypeptide, but also has endopeptidase activity.

Cofactor: Binds 1 calcium ion per subunit.

Subunit structure: Monomer. Ref.16

Subcellular location: Lysosome. Melanosome. Note: Identified by mass spectrometry in melanosome fractions from stage I to stage IV. Ref.9 Ref.12

Tissue specificity: Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.

Post-translational modification: Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.

Involvement in disease: Ceroid lipofuscinosis, neuronal, 2 (CLN2) [MIM:204500]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29

Sequence similarities: Contains 1 peptidase S53 domain.

Sequence caution: The sequence AAM08412.1 differs from that shown. Reason: Incorrectly indicated as originating from bovine.The sequence AAQ88866.1 differs from that shown. Reason: Frameshift at position 551.

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