Crystal Structure of the Precursor Form of Human Tripeptidyl-Peptidase 1

Crystal Structure and Autoactivation Pathway of the Precursor Form of Human Tripeptidyl-peptidase 1, the Enzyme Deficient in Late Infantile Ceroid Lipofuscinosis

horizontally deflecting and focusing crystal preceded by a vertically focusing mirror

Tripeptidyl-peptidase I, TPP-1, TPP-I, Tripeptidyl aminopeptidase, Lysosomal pepstatin- insensitive protease, LPIC, Cell growth-inhibiting gene 1 protein

SYSPEPDQRRTLPPGWVSLGRADPEEELSLTFALRQQNVERLSELVQAVSDPSSPQYGKYLTLENVADLVRPSPLTLHTV
QKWLLAAGAQKCHSVITQDFLTCWLSIRQAELLLPGAEFHHYVGGPTETHVVRSPHPYQLPQALAPHVDFVGGLHRFPPT
SSLRQRPEPQVTGTVGLHLGVTPSVIRKRYNLTSQDVGSGTSNNSQACAQFLEQYFHDSDLAQFMRLFGGNFAHQASVAR
VVGQQGRGRAGIEASLDVQYLMSAGANISTWVYSSPGRHEGQEPFLQWLMLLSNESALPHVHTVSYGDDEDSLSSAYIQR
VNTELMKAAARGLTLLFASGDSGAGCWSVSGRHQFRPTFPASSPYVTTVGGTSFQEPFLITNEIVDYISGGGFSNVFPRP
SYQEEAVTKFLSSSPHLPPSSYFNASGRAYPDVAALSDGYWVVSNRVPIPWVSGTSASTPVFGGILSLINEHRILSGRPP
LGFLNPRLYQQHGAGLFDVTRGCHESCLDEEVEGQGFCSGPGWDPVTGWGTPNFPALLKTLLNP

PEG 6000, citrate, pH 5.0, vapor diffusion, hanging drop, temperature 278K

HYDROGENS HAVE BEEN ADDED IN THE RIDING POSITIONS

protease, TPP1, sedolisin, Batten disease, LINCL, zymogen, prodomain, exopeptidase, endopeptidase, S53 family, cln2, catalytic triad, oxyanion hole, Disease mutation, Epilepsy, Glycoprotein, Hydrolase, Lysosome, Neuronal ceroid lipofuscinosis, Serine protease