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Reactivity | Human Mouse Rat |
Tested applications | WB IHC |
Recommended Dilution | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
Calculated MW | 61kDa |
Observed MW | Refer to Figures |
Immunogen | Recombinant protein of human TPP1 |
Storage Buffer | Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Synonym | CLN2; GIG1; LPIC; TPP-1; |
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.
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