Recombinant Human TPP1 Protein, GMP Grade
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| Cat#: |
TPP1-112THP |
| Common Name: |
TPP1 |
| Product Name: |
Recombinant Human TPP1 Protein, GMP Grade |
| Product Overview: |
Recombinant Human TPP1 Protein without tag was produced in an animal component free process under cGMP guidelines. |
| 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. |
| Species: |
Human |
| Bio-activity: |
30 mg/mL |
| Molecular Mass: |
59.0 Da (glycosylated) |
| AA Sequence: |
SYSPEPDQRRTLPPGWVSLGRADPEEELSLTFALRQQNVERLSELVQAVSDPSSPQYGKYLTLENVADLVRPSPLTLHTVQKWLLAAGAQKCHSVITQDFLTCWLSIRQAELLLPGAEFHHYVGGPTETHVVRSPHPYQLPQALAPHVDFVGGLHRFPPTSSLRQRPEPQVTGTVGLHLGVTPSVIRKRYNLTSQDVGSGTSNNSQACAQFLEQYFHDSDLAQFMRLFGGNFAHQASVARVVGQQGRGRAGIEASLDVQYLMSAGANISTWVYSSPGRHEGQEPFLQWLMLLSNESALPHVHTVSYGDDEDSLSSAYIQRVNTELMKAAARGLTLLFASGDSGAGCWSVSGRHQFRPTFPASSPYVTTVGGTSFQEPFLITNEIVDYISGGGFSNVFPRPSYQEEAVTKFLSSSPHLPPSSYFNASGRAYPDVAALSDGYWVVSNRVPIPWVSGTSASTPVFGGILSLINEHRILSGRPPLGFLNPRLYQQHGAGLFDVTRGCHESCLDEEVEGQGFCSGPGWDPVTGWGTPNFPALLKTLLNP |
| Endotoxin: |
<0.001 EU/μg of the peptide by the LAL method |
| Purity: |
> 99 % by SDS-PAGE and HPLC analysis |
| Applications: |
The product is a treatment for late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease to decelerate the progressive motor function decline in patients 3 years of age and older. CLN2 disease is a form of Batten disease, a rare inherited neurodegenerative disorder and is associated with seizures, ataxia, rapid loss of language and motor functions, blindness, and early death. It is caused by the lack the lysosomal enzyme tripeptidyl peptidase-1 (TPP1) and subsequent accumulation of lysosomal storage materials normally metabolized by this enzyme in the central nervous system. |
| Usage: |
Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) |
| Gene Name: |
TPP1 tripeptidyl peptidase I [ Homo sapiens (human) ] |
| Official Symbol: |
TPP1 |
| Synonyms: |
TPP1; tripeptidyl peptidase I; ceroid lipofuscinosis, neuronal 2, late infantile (Jansky Bielschowsky disease) , CLN2; tripeptidyl-peptidase 1; TPP I; tripeptidyl aminopeptidase; growth-inhibiting protein 1; cell growth-inhibiting gene 1 protein; lysosomal pepstatin insensitive protease; CLN2; GIG1; LPIC; TPP-1; MGC21297; |
| GeneID: |
1200 |
| mRNA Refseq: |
NM_000391 |
| Protein Refseq: |
NP_000382 |
| MIM: |
607998 |
| UniProt ID: |
O14773 |
Not For Human Consumption!
