##gff-version 3 P09936 UniProtKB Chain 1 220 . . . ID=PRO_0000211055;Note=Ubiquitin carboxyl-terminal hydrolase isozyme L1 P09936 UniProtKB Propeptide 221 223 . . . ID=PRO_0000414311;Note=Removed in mature form;Ontology_term=ECO:0000305;evidence=ECO:0000305 P09936 UniProtKB Region 5 10 . . . Note=Interaction with ubiquitin;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:20439756;Dbxref=PMID:20439756 P09936 UniProtKB Region 211 216 . . . Note=Interaction with ubiquitin;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:20439756;Dbxref=PMID:20439756 P09936 UniProtKB Active site 90 90 . . . Note=Nucleophile;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:20439756;Dbxref=PMID:20439756 P09936 UniProtKB Active site 161 161 . . . Note=Proton donor;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:20439756;Dbxref=PMID:20439756 P09936 UniProtKB Site 1 1 . . . Note=Susceptible to oxidation;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:14722078;Dbxref=PMID:14722078 P09936 UniProtKB Site 6 6 . . . Note=Susceptible to oxidation;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:14722078;Dbxref=PMID:14722078 P09936 UniProtKB Site 12 12 . . . Note=Susceptible to oxidation;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:14722078;Dbxref=PMID:14722078 P09936 UniProtKB Site 124 124 . . . Note=Susceptible to oxidation;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:14722078;Dbxref=PMID:14722078 P09936 UniProtKB Site 176 176 . . . Note=Important for enzyme activity;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8639624;Dbxref=PMID:8639624 P09936 UniProtKB Site 179 179 . . . Note=Susceptible to oxidation;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:14722078;Dbxref=PMID:14722078 P09936 UniProtKB Site 220 220 . . . Note=Susceptible to oxidation;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:14722078;Dbxref=PMID:14722078 P09936 UniProtKB Modified residue 125 125 . . . Note=Phosphoserine;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q00981 P09936 UniProtKB Lipidation 220 220 . . . Note=S-farnesyl cysteine;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:19261853;Dbxref=PMID:19261853 P09936 UniProtKB Natural variant 2 223 . . . ID=VAR_087898;Note=In SPG79A. Missing;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:35986737;Dbxref=PMID:35986737 P09936 UniProtKB Natural variant 7 7 . . . ID=VAR_070875;Note=In SPG79B%3B has decreased binding to ubiquitin and significantly decreased hydrolase activity compared to wild-type. E->A;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:23359680;Dbxref=dbSNP:rs397515634,PMID:23359680 P09936 UniProtKB Natural variant 18 18 . . . ID=VAR_015677;Note=It confers protection from oxidative stress when expressed at physiological levels in neuroblastoma cells and primary cortical neurons%3B loss of dimerization ability%3B impaired ligase activity. S->Y;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10203348,ECO:0000269|PubMed:11027850,ECO:0000269|PubMed:12408865,ECO:0000269|PubMed:12705903,ECO:0000269|PubMed:15048890,ECO:0000269|PubMed:16450370,ECO:0000269|PubMed:18411255,ECO:0000269|PubMed:21268678;Dbxref=dbSNP:rs5030732,PMID:10203348,PMID:11027850,PMID:12408865,PMID:12705903,PMID:15048890,PMID:16450370,PMID:18411255,PMID:21268678 P09936 UniProtKB Natural variant 25 223 . . . ID=VAR_087899;Note=In SPG79A. Missing;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:35986737;Dbxref=PMID:35986737 P09936 UniProtKB Natural variant 52 52 . . . ID=VAR_087900;Note=In SPG79A%3B uncertain significance. L->LL;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:35986737;Dbxref=PMID:35986737 P09936 UniProtKB Natural variant 93 93 . . . ID=VAR_015678;Note=In PARK5%3B impaired enzymatic hydrolase activity%3B has about a 50%25 reduction in catalytic activity compared to wild-type protein. I->M;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10454131,ECO:0000269|PubMed:12408865,ECO:0000269|PubMed:12705903,ECO:0000269|PubMed:23359680,ECO:0000269|PubMed:9774100;Dbxref=dbSNP:rs121917767,PMID:10454131,PMID:12408865,PMID:12705903,PMID:23359680,PMID:9774100 P09936 UniProtKB Natural variant 178 223 . . . ID=VAR_087901;Note=In SPG79A. Missing;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:35986737;Dbxref=PMID:35986737 P09936 UniProtKB Natural variant 178 178 . . . ID=VAR_078119;Note=In SPG79B%3B increased hydrolase activity%3B decreased protein abundance. R->Q;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:28007905;Dbxref=dbSNP:rs768996179,PMID:28007905 P09936 UniProtKB Natural variant 211 223 . . . ID=VAR_087902;Note=In SPG79A. Missing;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:35986737;Dbxref=PMID:35986737 P09936 UniProtKB Natural variant 216 216 . . . ID=VAR_078120;Note=In SPG79B%3B decreased protein abundance. A->D;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:28007905;Dbxref=dbSNP:rs1057519600,PMID:28007905 P09936 UniProtKB Mutagenesis 73 73 . . . Note=No effect on enzymatic parameters. Q->R;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8639624;Dbxref=PMID:8639624 P09936 UniProtKB Mutagenesis 90 90 . . . Note=Abolishes enzymatic activity. C->S;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:12705903,ECO:0000269|PubMed:20439756,ECO:0000269|PubMed:23359680,ECO:0000269|PubMed:25615526,ECO:0000269|PubMed:8639624;Dbxref=PMID:12705903,PMID:20439756,PMID:23359680,PMID:25615526,PMID:8639624 P09936 UniProtKB Mutagenesis 97 97 . . . Note=2-fold increase in affinity for ubiquitin ethyl ester%2C slight reduction in enzymatic activity. H->Q%2CN;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8639624;Dbxref=PMID:8639624 P09936 UniProtKB Mutagenesis 161 161 . . . Note=10000-fold decrease in enzymatic activity%3B no change in affinity for ubiquitin ethyl ester. H->D;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8639624;Dbxref=PMID:8639624 P09936 UniProtKB Mutagenesis 161 161 . . . Note=Abolishes enzymatic activity. H->K%2CQ%2CN%2CY;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8639624;Dbxref=PMID:8639624 P09936 UniProtKB Mutagenesis 176 176 . . . Note=6-fold decrease in affinity for ubiquitin ethyl ester%3B 97.5%25 decrease in enzymatic activity. D->N;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:8639624;Dbxref=PMID:8639624 P09936 UniProtKB Mutagenesis 204 204 . . . Note=Almost complete loss of activity. F->A;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:20439756;Dbxref=PMID:20439756 P09936 UniProtKB Helix 10 19 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 22 30 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 36 38 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 39 42 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 46 54 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 57 70 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Turn 71 74 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 86 88 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:2LEN P09936 UniProtKB Helix 90 100 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Turn 101 105 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 113 120 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Turn 121 123 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 126 134 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 137 147 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 160 168 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 171 175 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 179 181 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Beta strand 183 187 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 190 192 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 193 207 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ P09936 UniProtKB Helix 211 213 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:2LEN P09936 UniProtKB Beta strand 215 221 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:4JKJ