##gff-version 3 O95967 UniProtKB Signal peptide 1 23 . . . Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:23782690;Dbxref=PMID:23782690 O95967 UniProtKB Chain 24 443 . . . ID=PRO_0000007575;Note=EGF-containing fibulin-like extracellular matrix protein 2 O95967 UniProtKB Domain 36 81 . . . Note=EGF-like 1%3B atypical;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Domain 123 163 . . . Note=EGF-like 2%3B calcium-binding;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Domain 164 202 . . . Note=EGF-like 3%3B calcium-binding;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Domain 203 242 . . . Note=EGF-like 4%3B calcium-binding;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Domain 243 282 . . . Note=EGF-like 5%3B calcium-binding;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Domain 283 328 . . . Note=EGF-like 6%3B calcium-binding;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Site 87 88 . . . Note=Cleavage%3B by ELANE;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:27339457;Dbxref=PMID:27339457 O95967 UniProtKB Site 90 91 . . . Note=Cleavage%3B by MMP2%2C MMP3%2C MMP7%2C MMP9%2C MMP12;Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:23782690,ECO:0000269|PubMed:27339457;Dbxref=PMID:23782690,PMID:27339457 O95967 UniProtKB Site 92 93 . . . Note=Cleavage;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:23782690;Dbxref=PMID:23782690 O95967 UniProtKB Glycosylation 198 198 . . . Note=N-linked (GlcNAc...) asparagine;Ontology_term=ECO:0000255;evidence=ECO:0000255 O95967 UniProtKB Glycosylation 394 394 . . . Note=N-linked (GlcNAc...) asparagine;Ontology_term=ECO:0000255;evidence=ECO:0000255 O95967 UniProtKB Disulfide bond 58 121 . . . Ontology_term=ECO:0000255,ECO:0000269;evidence=ECO:0000255|PROSITE-ProRule:PRU00076,ECO:0000269|Ref.18 O95967 UniProtKB Disulfide bond 65 80 . . . Ontology_term=ECO:0000255,ECO:0000269;evidence=ECO:0000255|PROSITE-ProRule:PRU00076,ECO:0000269|Ref.18 O95967 UniProtKB Disulfide bond 71 109 . . . Ontology_term=ECO:0000255,ECO:0000269;evidence=ECO:0000255|PROSITE-ProRule:PRU00076,ECO:0000269|Ref.18 O95967 UniProtKB Disulfide bond 127 140 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 134 149 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 151 162 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 168 177 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 173 186 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 188 201 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 207 217 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 213 226 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 228 241 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 247 258 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 254 267 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 269 281 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 287 300 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 294 309 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Disulfide bond 315 327 . . . Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00076 O95967 UniProtKB Natural variant 57 57 . . . ID=VAR_027019;Note=In ARCL1B%3B does not affect secretion%3B decreased elastic fiber assembly%3B loss of interaction with collagen type IV trimer%2C FBN1%2C highly glycosylated form of LOX and LTBP1%3B Does not affect interaction with LOX when LOX is non-glycosylated%3B proteolyzed by PLG. E->K;Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:16685658,ECO:0000269|PubMed:27339457;Dbxref=dbSNP:rs119489101,PMID:16685658,PMID:27339457 O95967 UniProtKB Natural variant 126 126 . . . ID=VAR_084029;Note=In ARCL1B%3B slightly decreased protein abundance in patient dermal fibroblasts%3B increased transforming growth factor beta receptor signaling pathway in patient fibroblasts%3B strongly decreased secretion%3B decreased elastic fiber assembly%3B loss of interaction with collagen type IV trimer%2C FBN1 and LTBP1%3B decreased interaction with LOXL2%3B new fragments proteolyzed by ELANE%3B new one fragment proteolyzed by MMP2. E->K;Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:20389311,ECO:0000269|PubMed:27339457;Dbxref=PMID:20389311,PMID:27339457 O95967 UniProtKB Natural variant 126 126 . . . ID=VAR_084030;Note=In ARCL1B. E->V;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:20389311;Dbxref=PMID:20389311 O95967 UniProtKB Natural variant 203 203 . . . ID=VAR_084031;Note=In ARCL1B. D->A;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:22943132;Dbxref=PMID:22943132 O95967 UniProtKB Natural variant 227 227 . . . ID=VAR_084032;Note=In ARCL1B. R->C;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:22943132;Dbxref=PMID:22943132 O95967 UniProtKB Natural variant 259 259 . . . ID=VAR_027020;Note=I->V;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:10601734,ECO:0000269|PubMed:10982184,ECO:0000269|PubMed:12975309,ECO:0000269|PubMed:14702039,ECO:0000269|PubMed:15489334,ECO:0000269|PubMed:16303743,ECO:0000269|Ref.2;Dbxref=dbSNP:rs601314,PMID:10601734,PMID:10982184,PMID:12975309,PMID:14702039,PMID:15489334,PMID:16303743 O95967 UniProtKB Natural variant 267 267 . . . ID=VAR_067069;Note=In ARCL1B%3B loss of protein expression in patient dermal fibroblasts%3B increased transforming growth factor beta receptor signaling pathway in patient fibroblasts%3B loss of protein expression%3B strongly decreased secretion. C->Y;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:19664000,ECO:0000269|PubMed:20389311,ECO:0000269|PubMed:27339457;Dbxref=dbSNP:rs193302866,PMID:19664000,PMID:20389311,PMID:27339457 O95967 UniProtKB Natural variant 279 279 . . . ID=VAR_067070;Note=In ARCL1B%3B loss of secretion. R->C;Ontology_term=ECO:0000269,ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:17937443,ECO:0000269|PubMed:20389311,ECO:0000269|PubMed:27339457;Dbxref=dbSNP:rs119489102,PMID:17937443,PMID:20389311,PMID:27339457 O95967 UniProtKB Natural variant 397 397 . . . ID=VAR_084033;Note=In ARCL1B%3B increased N-glycosylation%3B introduced an extra O-glycosylation site%3B does not affect secretion%3B decreased elastic fiber assembly%3B decreased interaction with collagen type IV trimer%2C FBN1%2C ELN%2C LTBP1%2C LOXL1 and LOXL2%3B new fragments proteolyzed by ELANE. A->T;Ontology_term=ECO:0000269,ECO:0000269;evidence=ECO:0000269|PubMed:20389311,ECO:0000269|PubMed:27339457;Dbxref=PMID:20389311,PMID:27339457 O95967 UniProtKB Sequence conflict 5 5 . . . Note=A->T;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 24 27 . . . Note=SASP->APLA;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 44 51 . . . Note=EWDPDSQH->TQTAN;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 46 46 . . . Note=D->G;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 96 96 . . . Note=P->L;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 103 111 . . . Note=AQHPNPCPP->VNTQPLPT;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 294 294 . . . Note=C->W;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 354 356 . . . Note=RSV->AER;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Sequence conflict 355 355 . . . Note=S->R;Ontology_term=ECO:0000305;evidence=ECO:0000305 O95967 UniProtKB Beta strand 68 73 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:2KL7 O95967 UniProtKB Beta strand 78 82 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:2KL7 O95967 UniProtKB Beta strand 113 116 . . . Ontology_term=ECO:0007829;evidence=ECO:0007829|PDB:2KL7