ID:OS9_HUMAN DESCRIPTION: RecName: Full=Protein OS-9; AltName: Full=Amplified in osteosarcoma 9; Flags: Precursor; FUNCTION: Lectin which functions in endoplasmic reticulum (ER) quality control and ER-associated degradation (ERAD). May bind terminally misfolded non-glycosylated proteins as well as improperly folded glycoproteins, retain them in the ER, and possibly transfer them to the ubiquitination machinery and promote their degradation. Possible targets include TRPV4. SUBUNIT: Probably part of the HRD1 ubiquitin ligase complex composed at least of SYVN1/HRD1 and SEL1L with which it interacts directly. Through this complex it may interact with ERLEC1 and HSPA5. Interacts with DERL2. Interacts with HSP90B1 and CREB3. INTERACTION: Q9GZT9:EGLN1; NbExp=4; IntAct=EBI-1174342, EBI-1174818; Q16665:HIF1A; NbExp=9; IntAct=EBI-1174342, EBI-447269; SUBCELLULAR LOCATION: Endoplasmic reticulum lumen. TISSUE SPECIFICITY: Ubiquitously expressed. Found as well in all tumor cell lines analyzed, amplified in sarcomas. Highly expressed in osteosarcoma OSA-CL and rhabdomyosarcoma RH30 cell lines. Isoform 2 is the major isoform detected in all cell types examined. INDUCTION: Up-regulated in response to endoplasmic reticulum stress (at protein level). PTM: Intramolecular disulfide bonds. PTM: Isoform 1 and isoform 2 are N-glycosylated. SIMILARITY: Belongs to the OS-9 family. SIMILARITY: Contains 1 PRKCSH domain.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q13438
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006511 ubiquitin-dependent protein catabolic process GO:0006605 protein targeting GO:0006621 protein retention in ER lumen GO:0016567 protein ubiquitination GO:0030433 ER-associated ubiquitin-dependent protein catabolic process GO:0034976 response to endoplasmic reticulum stress GO:0055085 transmembrane transport GO:1904153 negative regulation of retrograde protein transport, ER to cytosol GO:1904380 endoplasmic reticulum mannose trimming
LF384692 - JP 2014500723-A/192195: Polycomb-Associated Non-Coding RNAs. AK303291 - Homo sapiens cDNA FLJ55400 complete cds, highly similar to Protein OS-9 precursor. AK291374 - Homo sapiens cDNA FLJ77871 complete cds, highly similar to Homo sapiens amplified in osteosarcoma, transcript variant 2, mRNA. AB002806 - Homo sapiens OS-9 mRNA, complete cds. U41635 - Human OS-9 precurosor mRNA, complete cds. AK294870 - Homo sapiens cDNA FLJ59883 complete cds, highly similar to Protein OS-9 precursor. AK293435 - Homo sapiens cDNA FLJ53302 complete cds, highly similar to Homo sapiens amplified in osteosarcoma (OS9), transcript variant 3, mRNA. AK129781 - Homo sapiens cDNA FLJ26270 fis, clone DMC06052, highly similar to Protein OS-9 precursor. AK296770 - Homo sapiens cDNA FLJ58173 complete cds, highly similar to Protein OS-9 precursor. AK304533 - Homo sapiens cDNA FLJ58567 complete cds, highly similar to Homo sapiens amplified in osteosarcoma (OS9), transcript variant 2, mRNA. AK298532 - Homo sapiens cDNA FLJ54114 complete cds, highly similar to Homo sapiens amplified in osteosarcoma (OS9), transcript variant 2, mRNA. AK130733 - Homo sapiens cDNA FLJ27223 fis, clone SYN04785. AK303858 - Homo sapiens cDNA FLJ53693 complete cds, highly similar to Homo sapiens amplified in osteosarcoma (OS9), transcript variant 2, mRNA. BC000532 - Homo sapiens osteosarcoma amplified 9, endoplasmic reticulum associated protein, mRNA (cDNA clone MGC:8691 IMAGE:2964645), complete cds. BC007254 - Homo sapiens osteosarcoma amplified 9, endoplasmic reticulum associated protein, mRNA (cDNA clone MGC:15510 IMAGE:3027883), complete cds. LF324134 - JP 2014500723-A/131637: Polycomb-Associated Non-Coding RNAs. BC023513 - Homo sapiens osteosarcoma amplified 9, endoplasmic reticulum associated protein, mRNA (cDNA clone MGC:12783 IMAGE:4123782), complete cds. CU674262 - Synthetic construct Homo sapiens gateway clone IMAGE:100017739 5' read OS9 mRNA. CU675585 - Synthetic construct Homo sapiens gateway clone IMAGE:100017430 5' read OS9 mRNA. CR456791 - Homo sapiens full open reading frame cDNA clone RZPDo834H034D for gene OS-9, amplified in osteosarcoma; complete cds, incl. stopcodon. AB590116 - Synthetic construct DNA, clone: pFN21AB0404, Homo sapiens OS9 gene for osteosarcoma amplified 9, endoplasmic reticulum lectin, without stop codon, in Flexi system. DQ892759 - Synthetic construct clone IMAGE:100005389; FLH189426.01X; RZPDo839F0174D amplified in osteosarcoma (OS9) gene, encodes complete protein. DQ896004 - Synthetic construct Homo sapiens clone IMAGE:100010464; FLH189422.01L; RZPDo839F0164D amplified in osteosarcoma (OS9) gene, encodes complete protein. KJ893179 - Synthetic construct Homo sapiens clone ccsbBroadEn_02573 OS9 gene, encodes complete protein. KJ898320 - Synthetic construct Homo sapiens clone ccsbBroadEn_07714 OS9 gene, encodes complete protein. LF324133 - JP 2014500723-A/131636: Polycomb-Associated Non-Coding RNAs. LF324131 - JP 2014500723-A/131634: Polycomb-Associated Non-Coding RNAs. LF324130 - JP 2014500723-A/131633: Polycomb-Associated Non-Coding RNAs. AL137691 - Homo sapiens mRNA; cDNA DKFZp434P172 (from clone DKFZp434P172). BC000476 - Homo sapiens cDNA clone IMAGE:2961642, **** WARNING: chimeric clone ****. LF324129 - JP 2014500723-A/131632: Polycomb-Associated Non-Coding RNAs. LF208924 - JP 2014500723-A/16427: Polycomb-Associated Non-Coding RNAs. LF324128 - JP 2014500723-A/131631: Polycomb-Associated Non-Coding RNAs. LF324125 - JP 2014500723-A/131628: Polycomb-Associated Non-Coding RNAs. EF036505 - Homo sapiens OS-9 precursor, mRNA, partial cds. BC006506 - Homo sapiens osteosarcoma amplified 9, endoplasmic reticulum associated protein, mRNA (cDNA clone IMAGE:2961147), partial cds. LF324122 - JP 2014500723-A/131625: Polycomb-Associated Non-Coding RNAs. LF324119 - JP 2014500723-A/131622: Polycomb-Associated Non-Coding RNAs. LF324117 - JP 2014500723-A/131620: Polycomb-Associated Non-Coding RNAs. LF324116 - JP 2014500723-A/131619: Polycomb-Associated Non-Coding RNAs. U09408 - Human clone OS-9 chromosome 12q13 mRNA sequence. LF324114 - JP 2014500723-A/131617: Polycomb-Associated Non-Coding RNAs. JD021303 - Sequence 2327 from Patent EP1572962. JD032297 - Sequence 13321 from Patent EP1572962. LF324113 - JP 2014500723-A/131616: Polycomb-Associated Non-Coding RNAs. LF324111 - JP 2014500723-A/131614: Polycomb-Associated Non-Coding RNAs. LF324110 - JP 2014500723-A/131613: Polycomb-Associated Non-Coding RNAs. LF324109 - JP 2014500723-A/131612: Polycomb-Associated Non-Coding RNAs. JD382358 - Sequence 363382 from Patent EP1572962. JD415775 - Sequence 396799 from Patent EP1572962. JD343704 - Sequence 324728 from Patent EP1572962. JD188516 - Sequence 169540 from Patent EP1572962. JD527985 - Sequence 509009 from Patent EP1572962. JD427569 - Sequence 408593 from Patent EP1572962. JD220766 - Sequence 201790 from Patent EP1572962. JD544910 - Sequence 525934 from Patent EP1572962. JD329528 - Sequence 310552 from Patent EP1572962. JD321504 - Sequence 302528 from Patent EP1572962. JD245302 - Sequence 226326 from Patent EP1572962. JD153955 - Sequence 134979 from Patent EP1572962. JD176677 - Sequence 157701 from Patent EP1572962. JD289800 - Sequence 270824 from Patent EP1572962. JD403375 - Sequence 384399 from Patent EP1572962. JD093921 - Sequence 74945 from Patent EP1572962. JD555074 - Sequence 536098 from Patent EP1572962. LF324108 - JP 2014500723-A/131611: Polycomb-Associated Non-Coding RNAs. JD363089 - Sequence 344113 from Patent EP1572962. MA620269 - JP 2018138019-A/192195: Polycomb-Associated Non-Coding RNAs. MA559711 - JP 2018138019-A/131637: Polycomb-Associated Non-Coding RNAs. MA559710 - JP 2018138019-A/131636: Polycomb-Associated Non-Coding RNAs. MA559708 - JP 2018138019-A/131634: Polycomb-Associated Non-Coding RNAs. MA559707 - JP 2018138019-A/131633: Polycomb-Associated Non-Coding RNAs. MA559706 - JP 2018138019-A/131632: Polycomb-Associated Non-Coding RNAs. MA559705 - JP 2018138019-A/131631: Polycomb-Associated Non-Coding RNAs. MA559702 - JP 2018138019-A/131628: Polycomb-Associated Non-Coding RNAs. MA559699 - JP 2018138019-A/131625: Polycomb-Associated Non-Coding RNAs. MA559696 - JP 2018138019-A/131622: Polycomb-Associated Non-Coding RNAs. MA559694 - JP 2018138019-A/131620: Polycomb-Associated Non-Coding RNAs. MA559693 - JP 2018138019-A/131619: Polycomb-Associated Non-Coding RNAs. MA559691 - JP 2018138019-A/131617: Polycomb-Associated Non-Coding RNAs. MA559690 - JP 2018138019-A/131616: Polycomb-Associated Non-Coding RNAs. MA559688 - JP 2018138019-A/131614: Polycomb-Associated Non-Coding RNAs. MA559687 - JP 2018138019-A/131613: Polycomb-Associated Non-Coding RNAs. MA559686 - JP 2018138019-A/131612: Polycomb-Associated Non-Coding RNAs. MA559685 - JP 2018138019-A/131611: Polycomb-Associated Non-Coding RNAs. MA444501 - JP 2018138019-A/16427: Polycomb-Associated Non-Coding RNAs.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein Q13438 (Reactome details) participates in the following event(s):
R-HSA-5362437 C-terminal Hh fragments are bound by lectins R-HSA-5362450 Hh processing variants bind lectins R-HSA-5362459 VCP-catalyzed ATP hydrolysis promotes the translocation of Hh-C into the cytosol R-HSA-5387389 Hh processing variants are translocated to the cytosol in a VCP-dependent manner R-HSA-8867288 OS9:SEL1:ERAD E3 ligase:DERL2 ubiquitinates unfolded protein:(GlcNAc)2 (Man)9-5 R-HSA-1022127 OS9:SEL1:ERAD E3 ligase:DERL2 transports Ub-unfolded protein:(GlcNAc)2 (Man)9-5 from ERQC to cytosol R-HSA-5387386 Hh processing variants are recruited to SEL1:SYVN at the ER membrane R-HSA-5362412 SYVN1 ubiquitinates Hh C-terminal fragments R-HSA-5362441 C-terminal Hh fragments are recruited to SEL1:SYVN1 at the ER membrane R-HSA-8866551 CFTR binds components of the ERAD machinery for ubiquitination and degradation R-HSA-8866857 CFTR F508del binds components of the ERAD machinery for ubiquitination and degradation R-HSA-8866542 VCP-catalyzed ATP hydrolysis promotes the translocation of misfolded CFTR into the cytosol R-HSA-8866854 VCP-catalyzed ATP hydrolysis promotes the translocation of CFTR F508del into the cytosol R-HSA-5483238 Hh processing variants are ubiquitinated R-HSA-8866546 RNF5 and RNF185 ubiquitinate misfolded CFTR R-HSA-8866856 RNF5 and RNF185 ubiquitinate CFTR F508del R-HSA-5358346 Hedgehog ligand biogenesis R-HSA-5362768 Hh mutants that don't undergo autocatalytic processing are degraded by ERAD R-HSA-901032 ER Quality Control Compartment (ERQC) R-HSA-382556 ABC-family proteins mediated transport R-HSA-5678895 Defective CFTR causes cystic fibrosis R-HSA-5358351 Signaling by Hedgehog R-HSA-5387390 Hh mutants abrogate ligand secretion R-HSA-901042 Calnexin/calreticulin cycle R-HSA-382551 Transport of small molecules R-HSA-5619084 ABC transporter disorders R-HSA-162582 Signal Transduction R-HSA-5663202 Diseases of signal transduction R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle R-HSA-5619115 Disorders of transmembrane transporters R-HSA-1643685 Disease R-HSA-446203 Asparagine N-linked glycosylation R-HSA-597592 Post-translational protein modification R-HSA-392499 Metabolism of proteins
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
