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Gene Review

RPN2  -  ribophorin II

Homo sapiens

Synonyms: Dolichyl-diphosphooligosaccharide--protein glycosyltransferase 63 kDa subunit, Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2, RIBIIR, RPN-II, RPNII, ...
 
 
 

Summary

This gene encodes a type I integral membrane protein found only in the rough endoplasmic reticulum. The encoded protein is part of an N-oligosaccharyl transferase complex that links high mannose oligosaccharides to asparagine residues found in the Asn-X-Ser/Thr consensus motif of nascent polypeptide chains. This protein is similar in sequence to the yeast oligosaccharyl transferase subunit SWP1. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]

 

Function

Essential subunit of N-oligosaccharyl transferase enzyme which catalyzes the transfer of a high mannose oligosaccharide from a lipid-linked oligosaccharide donor to an asparagine residue within an Asn-X-Ser/Thr consensus motif in nascent polypeptide chains.

 

Disease relevance of RPN2

 

High impact information on RPN2

  • In the case of ribophorin II, it is completely resistant to such proteolysis which is consistent with its luminal disposition and fairly hydrophobic C terminus.(ABSTRACT TRUNCATED AT 250 WORDS)[2]
  • Ribophorin I was 85% resistant and ribophorin II 100% resistant to the levels of protease needed to totally eliminate ribosome binding [3].
  • The myeloid tumor suppressor gene was localized to an 18-centimorgan interval (approximately equal to 13 Mb) between RPN2 and D20S17 [4].
  • Dinucleotide repeat polymorphism at the human ribophorin II locus (RPN2) on chromosome 20q [5].
  • What curves alpha-solenoids? Evidence for an alpha-helical toroid structure of Rpn1 and Rpn2 proteins of the 26 S proteasome [6].
 

Biological context of RPN2

 

Anatomical context of RPN2

 

Other interactions of RPN2

  • In the case of the Tac chimera containing only the luminal domain of RII, which by itself exits from the ER and is rapidly degraded, it is retained in the ER and becomes stabilized when coexpressed with OST48 [13].
  • A series of known genes which have not previously been reported to be overexpressed in cancer were also recovered: Hsc70, PBEF, ribophorin II and Ese-3B [14].
 

Analytical, diagnostic and therapeutic context of RPN2

  • N-terminal sequence analysis identified the proteins as ribophorin I, ribophorin II (doublet), and a 50-kDa homologue of Wbp1, a yeast protein essential for N-glycosylation [15].
  • Based on the length polymorphism and sequence diversities of the PTP and SWP-1 genes, two simple PCR tests were developed to differentiate E. cuniculi in clinical samples [10].

References

  1. Lymphovascular invasion is associated with poor survival in gastric cancer: an application of gene-expression and tissue array techniques. Dicken, B.J., Graham, K., Hamilton, S.M., Andrews, S., Lai, R., Listgarten, J., Jhangri, G.S., Saunders, L.D., Damaraju, S., Cass, C. Ann. Surg. (2006) [Pubmed]
  2. Human ribophorins I and II: the primary structure and membrane topology of two highly conserved rough endoplasmic reticulum-specific glycoproteins. Crimaudo, C., Hortsch, M., Gausepohl, H., Meyer, D.I. EMBO J. (1987) [Pubmed]
  3. Characterization of secretory protein translocation: ribosome-membrane interaction in endoplasmic reticulum. Hortsch, M., Avossa, D., Meyer, D.I. J. Cell Biol. (1986) [Pubmed]
  4. A yeast artificial chromosome-based map of the region of chromosome 20 containing the diabetes-susceptibility gene, MODY1, and a myeloid leukemia related gene. Stoffel, M., Le Beau, M.M., Espinosa, R., Bohlander, S.F., Le Paslier, D., Cohen, D., Xiang, K.S., Cox, N.J., Fajans, S.S., Bell, C.I. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  5. Dinucleotide repeat polymorphism at the human ribophorin II locus (RPN2) on chromosome 20q. Stoffel, M., Xiang, K., Bell, G.I. Hum. Mol. Genet. (1992) [Pubmed]
  6. What curves alpha-solenoids? Evidence for an alpha-helical toroid structure of Rpn1 and Rpn2 proteins of the 26 S proteasome. Kajava, A.V. J. Biol. Chem. (2002) [Pubmed]
  7. A detailed deletion map of chromosome 20 in human oral squamous cell carcinoma. Imai, F.L., Uzawa, K., Miyakawa, A., Shiiba, M., Tanzawa, H. Int. J. Mol. Med. (2001) [Pubmed]
  8. Mapping of the ribophorin II (RPN II) gene to human chromosome 20q12-q13.1 by in-situ hybridization. Löffler, C., Rao, V.V., Hansmann, I. Hum. Genet. (1991) [Pubmed]
  9. Serodiagnostic studies in an immunocompetent individual infected with Encephalitozoon cuniculi. van Gool, T., Biderre, C., Delbac, F., Wentink-Bonnema, E., Peek, R., Vivarès, C.P. J. Infect. Dis. (2004) [Pubmed]
  10. Genotyping Encephalitozoon cuniculi by multilocus analyses of genes with repetitive sequences. Xiao, L., Li, L., Visvesvara, G.S., Moura, H., Didier, E.S., Lal, A.A. J. Clin. Microbiol. (2001) [Pubmed]
  11. Reassessment of the subcellular localization of p63. Schweizer, A., Rohrer, J., Slot, J.W., Geuze, H.J., Kornfeld, S. J. Cell. Sci. (1995) [Pubmed]
  12. Membranous complexes characteristic of melanocytes derived from patients with Hermansky-Pudlak syndrome type 1 are macroautophagosomal entities of the lysosomal compartment. Smith, J.W., Koshoffer, A., Morris, R.E., Boissy, R.E. Pigment Cell Res. (2005) [Pubmed]
  13. Retention of subunits of the oligosaccharyltransferase complex in the endoplasmic reticulum. Fu, J., Kreibich, G. J. Biol. Chem. (2000) [Pubmed]
  14. A profile of differentially expressed genes in primary colorectal cancer using suppression subtractive hybridization. Hufton, S.E., Moerkerk, P.T., Brandwijk, R., de Bruïne, A.P., Arends, J.W., Hoogenboom, H.R. FEBS Lett. (1999) [Pubmed]
  15. Interleukin-2 induces N-glycosylation in T-cells: characterization of human lymphocyte oligosaccharyltransferase. Kumar, V., Heinemann, F.S., Ozols, J. Biochem. Biophys. Res. Commun. (1998) [Pubmed]
 
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