Osteosarcoma Amplified 9 (OS9)

ERLEC2; Amplified In Osteosarcoma; Endoplasmic Reticulum Lectin 2; Erlectin 2

SPN	U160
HGNC	16994
MGI	1924301
PubMed	21523322
RGD	1359574
UniProt	Q13438
Wiki	OS9_(gene

Protein OS-9 is a protein that is highly expressed in osteosarcomas.This protein binds to the hypoxia-inducible factor 1 (HIF-1), a key regulator of the hypoxic response and angiogenesis, and promotes the degradation of one of its subunits. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

The deduced 667-amino acid protein has a calculated molecular mass of 75.6 kD. OS9 contains an N-terminal leader peptide, a 211-amino acid hydrophilic region with 8 cysteines, and an acidic region with multiple predicted hydrophilic helices. It also has a putative nuclear targeting signal. The hydrophilic region of OS9 is highly conserved in human, S. cerevisiae, and C. elegans, with complete conservation of the 8 cysteines.

Reference

"Complete sequence analysis of a gene (OS-9) ubiquitously expressed in human tissues and amplified in sarcomas."
Mol. Carcinog. 15:270-275(1996) [PubMed] [Europe PMC] [Abstract]

"Genomic organization of the OS-9 gene amplified in human sarcomas."
J. Biochem. 122:1190-1195(1997) [PubMed] [Europe PMC] [Abstract]

"Cloning and characterization of three isoforms of OS-9 cDNA and expression of the OS-9 gene in various human tumor cell lines."
J. Biochem. 123:876-882(1998) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The finished DNA sequence of human chromosome 12."
Nature 440:346-351(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Transcript mapping in a 46-kb sequenced region at the core of 12q13.3 amplification in human cancers."
Genomics 42:295-301(1997) [PubMed] [Europe PMC] [Abstract]

"OS-9 regulates the transit and polyubiquitination of TRPV4 in the endoplasmic reticulum."
J. Biol. Chem. 282:36561-36570(2007) [PubMed] [Europe PMC] [Abstract]

"A dual task for the Xbp1-responsive OS-9 variants in the mammalian endoplasmic reticulum: inhibiting secretion of misfolded protein conformers and enhancing their disposal."
J. Biol. Chem. 283:16446-16454(2008) [PubMed] [Europe PMC] [Abstract]

"Human XTP3-B forms an endoplasmic reticulum quality control scaffold with the HRD1-SEL1L ubiquitin ligase complex and BiP."
J. Biol. Chem. 283:20914-20924(2008) [PubMed] [Europe PMC] [Abstract]

"OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD."
Nat. Cell Biol. 10:272-282(2008) [PubMed] [Europe PMC] [Abstract]

"Human OS-9, a lectin required for glycoprotein endoplasmic reticulum-associated degradation, recognizes mannose-trimmed N-glycans."
J. Biol. Chem. 284:17061-17068(2009) [PubMed] [Europe PMC] [Abstract]

"Mammalian OS-9 is upregulated in response to endoplasmic reticulum stress and facilitates ubiquitination of misfolded glycoproteins."
J. Mol. Biol. 385:1032-1042(2009) [PubMed] [Europe PMC] [Abstract]

"DC-STAMP interacts with ER-resident transcription factor LUMAN which becomes activated during DC maturation."
Mol. Immunol. 47:1963-1973(2010) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Structural basis for oligosaccharide recognition of misfolded glycoproteins by OS-9 in ER-associated degradation."
Mol. Cell 40:905-916(2010) [PubMed] [Europe PMC] [Abstract]