{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T21:39:29Z","timestamp":1770500369779,"version":"3.49.0"},"reference-count":80,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[1999,7,1]],"date-time":"1999-07-01T00:00:00Z","timestamp":930787200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nat Med"],"published-print":{"date-parts":[[1999,7]]},"DOI":"10.1038\/10466","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T08:43:33Z","timestamp":1027673013000},"page":"745-751","source":"Crossref","is-referenced-by-count":222,"title":["Integration of endoplasmic reticulum signaling in health and disease"],"prefix":"10.1038","volume":"5","author":[{"given":"Meir","family":"Aridor","sequence":"first","affiliation":[]},{"given":"William E.","family":"Balch","sequence":"additional","affiliation":[]}],"member":"297","published-online":{"date-parts":[[1999,7,1]]},"reference":[{"key":"BFnm0799745_CR1","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1016\/S0092-8674(00)80930-7","volume":"92","author":"KE Matlack","year":"1998","unstructured":"Matlack, K.E., Mothes, W. & Rapoport, T.A. Protein translocation: tunnel vision. Cell 92, 381\u2013390 (1998).","journal-title":"Cell"},{"key":"BFnm0799745_CR2","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1016\/S0959-440X(98)80148-6","volume":"8","author":"ES Trombetta","year":"1998","unstructured":"Trombetta, E.S. & Helenius, A. Lectins as chaperones in glycoprotein folding. Curr. Opin. Struct. Biol. 8, 587\u2013592 (1998).","journal-title":"Curr. Opin. Struct. Biol"},{"key":"BFnm0799745_CR3","doi-asserted-by":"publisher","first-page":"5861","DOI":"10.1074\/jbc.274.9.5861","volume":"274","author":"Y Liu","year":"1999","unstructured":"Liu, Y., Choudhury, P., Cabral, C.M. & Sifers, R.N. Oligosaccharide modification in the early secretory pathway directs the selection of a misfolded glycoprotein for degradation by the proteasome. J. Biol. Chem. 274, 5861\u20135867 (1999).","journal-title":"J. Biol. Chem"},{"key":"BFnm0799745_CR4","doi-asserted-by":"publisher","first-page":"523","DOI":"10.1016\/0955-0674(95)80009-3","volume":"7","author":"C Hammond","year":"1995","unstructured":"Hammond, C. & Helenius, A. Quality control in the secretory pathway. Curr. Opin. Cell Biol. 7, 523\u2013 529 (1995).","journal-title":"Curr. Opin. Cell Biol."},{"key":"BFnm0799745_CR5","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1146\/annurev.cellbio.14.1.459","volume":"14","author":"R Chapman","year":"1998","unstructured":"Chapman, R., Sidrauski, C. & Walter, P. Intracellular signaling from the endoplasmic reticulum to the nucleus. Annu. Rev. Cell. Dev. Biol. 14, 459\u2013485 (1998).","journal-title":"Annu. Rev. Cell. Dev. Biol"},{"key":"BFnm0799745_CR6","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1016\/S0962-8924(96)10050-7","volume":"7","author":"HL Pahl","year":"1997","unstructured":"Pahl, H.L. & Baeuerle, P.A. Endoplasmic reticulum induced signal transduction and gene expression. Trends Cell Biol. 7, 50\u201355 (1997).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR7","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1038\/16729","volume":"397","author":"HP Harding","year":"1999","unstructured":"Harding, H.P., Zhang, Y. & Ron, D. Protein translation and folding are coupled by an endoplasmic-reticulum-resident kinase. Nature 397, 271\u2013 274 (1999).","journal-title":"Nature"},{"key":"BFnm0799745_CR8","first-page":"63","volume":"22","author":"HL Pahl","year":"1997","unstructured":"Pahl, H.L. & Baeuerle, P.A. The ER-overload response: activation of NF-kB. Trends Cell Biol. 22 63\u2013 67 (1997).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR9","doi-asserted-by":"publisher","first-page":"1347","DOI":"10.1126\/science.272.5266.1347","volume":"272","author":"XZ Wang","year":"1996","unstructured":"Wang, X.Z. & Ron, D. Stress-induced phosphorylation and activation of the transcription factor CHOP (Gadd153) by p38 MAP kinase. Science 272, 1347\u20131349 (1996).","journal-title":"Science"},{"key":"BFnm0799745_CR10","doi-asserted-by":"crossref","first-page":"4654","DOI":"10.1002\/j.1460-2075.1995.tb00147.x","volume":"14","author":"N Batchvarova","year":"1995","unstructured":"Batchvarova, N., Wang, X.Z. & Ron, D. Inhibition of adipogenesis by the stress-induced protein CHOP (Gadd153). EMBO J. 14, 4654\u20134661 (1995).","journal-title":"EMBO J."},{"key":"BFnm0799745_CR11","doi-asserted-by":"publisher","first-page":"982","DOI":"10.1101\/gad.12.7.982","volume":"12","author":"H Zinszner","year":"1998","unstructured":"Zinszner, H. et al. CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum. Genes Dev. 12, 982\u2013995 (1998).","journal-title":"Genes Dev."},{"key":"BFnm0799745_CR12","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1016\/0962-8924(96)10027-1","volume":"6","author":"M Aridor","year":"1996","unstructured":"Aridor, M. & Balch, W.E. Principles of selective transport: coat complexes hold the key. Trends Cell Biol. 6, 315\u2013320 (1996).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR13","doi-asserted-by":"publisher","first-page":"4389","DOI":"10.1074\/jbc.274.7.4389","volume":"274","author":"M Aridor","year":"1999","unstructured":"Aridor, M., Bannykh, S.I., Rowe, T. & Balch, W.E. Cargo can modulate COPII vesicle formation from the endoplasmic reticulum. J. Biol. Chem. 274, 4389\u20134399 (1999).","journal-title":"J. Biol. Chem"},{"key":"BFnm0799745_CR14","doi-asserted-by":"publisher","first-page":"1526","DOI":"10.1126\/science.271.5255.1526","volume":"271","author":"R Schekman","year":"1996","unstructured":"Schekman, R. & Orci, L. Coat proteins and vesicle budding. Science 271, 1526\u20131533 (1996).","journal-title":"Science"},{"key":"BFnm0799745_CR15","doi-asserted-by":"publisher","first-page":"698","DOI":"10.1126\/science.281.5377.698","volume":"281","author":"S Springer","year":"1998","unstructured":"Springer, S. & Schekman, S. Nucleation of COPII vesicular coat complex by endoplasmic reticulum to Golgi vesicle SNAREs. Science 281, 698\u2013700 (1998).","journal-title":"Science"},{"key":"BFnm0799745_CR16","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1083\/jcb.141.1.61","volume":"141","author":"M Aridor","year":"1998","unstructured":"Aridor, M., Weissman, J., Bannykh, S., Nouffer, C. & Balch, W.E. Cargo selection by the COPII budding machinery during export from the endoplasmic reticulum. J. Cell Biol. 141, 61\u201370 (1998).","journal-title":"J. Cell Biol."},{"key":"BFnm0799745_CR17","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1083\/jcb.135.1.19","volume":"135","author":"SI Bannykh","year":"1996","unstructured":"Bannykh, S.I., Rowe, T. & Balch, W.E. Organization of endoplasmic reticulum export complexes. J. Cell Biol. 135, 19\u2013 35 (1996).","journal-title":"J. Cell Biol."},{"key":"BFnm0799745_CR18","doi-asserted-by":"publisher","first-page":"723","DOI":"10.1016\/0092-8674(90)90483-U","volume":"61","author":"CA Kaiser","year":"1990","unstructured":"Kaiser, C.A. & Schekman, R. Distinct sets of SEC genes govern transport vesicle formation and fusion early in the secretory pathway. Cell 61, 723\u2013733 (1990).","journal-title":"Cell"},{"key":"BFnm0799745_CR19","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1093\/oxfordjournals.jbchem.a022249","volume":"125","author":"Y Saito","year":"1999","unstructured":"Saito, Y., Yamanushi, T., Oka, T. & Nakano, A. Identification of SEC12, SED4, truncated SEC16 and EKS1\/HRD3 as multicopy suppressors of ts mutants of Sar1 GTPase. J. Biochem. (Tokyo) 125, 130\u2013137 (1999).","journal-title":"J. Biochem. (Tokyo)"},{"key":"BFnm0799745_CR20","doi-asserted-by":"publisher","first-page":"402","DOI":"10.1006\/abbi.1993.1368","volume":"304","author":"L Zhen","year":"1993","unstructured":"Zhen, L., Baumann, H., Novak, E.K. & Swank, R.T. The signal for retention of the egasyn-glucuronidase complex within the endoplasmic reticulum. Arch. Biochem. 304, 402\u2013 414 (1993).","journal-title":"Arch. Biochem."},{"key":"BFnm0799745_CR21","first-page":"456S","volume":"129","author":"GS Shelness","year":"1999","unstructured":"Shelness, G.S., Ingram, M.F., Huang, X.F. & DeLozier, J.A. Apolipoprotein B in the rough endoplasmic reticulum: translation, translocation and the initiation of lipoprotein assembly. J. Nutr. 129, 456S\u2013462S (1999).","journal-title":"J. Nutr"},{"key":"BFnm0799745_CR22","doi-asserted-by":"crossref","first-page":"4886","DOI":"10.1002\/j.1460-2075.1994.tb06816.x","volume":"13","author":"EK Baker","year":"1994","unstructured":"Baker, E.K., Colley, N.J. & Zuker, C.S. The cyclophilin homolog NinaA functions as a chaperone, forming a stable complex in vivo with its protein target rhodopsin. EMBO J. 13, 4886\u20134895 (1994).","journal-title":"EMBO J."},{"key":"BFnm0799745_CR23","doi-asserted-by":"publisher","first-page":"272","DOI":"10.1016\/S0962-8924(98)01283-5","volume":"8","author":"G Bu","year":"1998","unstructured":"Bu, G. & Schwartz, A.L. RAP, a novel type of ER chaperone. Trends Cell Biol. 8, 272\u2013 276 (1998).","journal-title":"Trends Cell Biol"},{"key":"BFnm0799745_CR24","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1038\/34438","volume":"391","author":"MJ Kuehn","year":"1998","unstructured":"Kuehn, M.J., Herrmann, M. & Schekman, R. COPII-cargo interactions direct protein sorting into ER-derived transport vesicles. Nature 391, 187\u2013190 (1998).","journal-title":"Nature"},{"key":"BFnm0799745_CR25","doi-asserted-by":"publisher","first-page":"556","DOI":"10.1126\/science.277.5325.556","volume":"277","author":"N Nishimura","year":"1997","unstructured":"Nishimura, N. & Balch, W.E. A di-acidic signal required for selective export from the endoplasmic reticulum. Science 277, 556\u2013558 (1997).","journal-title":"Science"},{"key":"BFnm0799745_CR26","doi-asserted-by":"publisher","first-page":"15937","DOI":"10.1074\/jbc.274.22.15937","volume":"274","author":"N Nishimura","year":"1999","unstructured":"Nishimura, N. et al. A di-acidic (DXE) code directs concentration of cargo during export from the endoplasmic reticulum. J. Biol. Chem. 274, 15937\u201315946 (1999).","journal-title":"J. Biol. Chem"},{"key":"BFnm0799745_CR27","doi-asserted-by":"publisher","first-page":"24739","DOI":"10.1074\/jbc.272.40.24739","volume":"272","author":"K Fiedler","year":"1997","unstructured":"Fiedler, K. & Rothman, J.E. Sorting determinants in the transmembrane domain of p24 proteins. J. Biol. Chem. 272, 24739\u201324742 (1997).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR28","doi-asserted-by":"publisher","first-page":"31801","DOI":"10.1074\/jbc.272.50.31801","volume":"272","author":"F Kappeler","year":"1997","unstructured":"Kappeler, F., Klopfenstein, D.R., Foguet, M., Paccaud, J.P. & Hauri, H.P. The recycling of ERGIC-53 in the early secretory pathway. ERGIC-53 carries a cytosolic endoplasmic reticulum-exit determinant interacting with COPII. J. Biol. Chem. 272, 31801\u201331808 (1997).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR29","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1016\/S0962-8924(97)01184-7","volume":"8","author":"S Bannykh","year":"1998","unstructured":"Bannykh, S., Nishimura, N. & Balch, W.E. Getting into the Golgi. Trends Cell Biol. 8, 21\u201325 (1998).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR30","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1016\/S0092-8674(00)81146-0","volume":"93","author":"WC Nichols","year":"1998","unstructured":"Nichols, W.C. et al. Mutations in the ER-Golgi intermediate compartment protein ERGIC-53 cause combined deficiency of coagulation factors V and VIII. Cell 93, 61\u201370 (1998).","journal-title":"Cell"},{"key":"BFnm0799745_CR31","doi-asserted-by":"publisher","first-page":"5048","DOI":"10.1074\/jbc.272.8.5048","volume":"272","author":"AL White","year":"1997","unstructured":"White, A.L., Guerra, B. & Lanford, R.E. Influence of allelic variation on apolipoprotein (a) folding in the endoplasmic reticulum. J. Biol. Chem. 272, 5048\u20135055 (1997).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR32","first-page":"173","volume":"19","author":"PS Kim","year":"1998","unstructured":"Kim, P.S. & Arvan, P. Endocrinopathies in the family of endoplasmic reticulum (ER) storage diseases: disorders of protein trafficking and the role of ER molecular chaperones. Endocr. Rev. 19, 173\u2013202 (1998).","journal-title":"Endocr. Rev."},{"key":"BFnm0799745_CR33","doi-asserted-by":"crossref","first-page":"24496","DOI":"10.1016\/S0021-9258(19)51111-5","volume":"269","author":"S Macintyre","year":"1994","unstructured":"Macintyre, S., Samols, D. & Dailey, P. Two carboxylesterases bind C-reactive protein within the endoplasmic reticulum and regulate its secretion during the acute phase response. J. Biol. Chem. 269, 24496\u2013 24503 (1994).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR34","doi-asserted-by":"publisher","first-page":"1056","DOI":"10.1073\/pnas.95.3.1056","volume":"95","author":"T Kuwana","year":"1998","unstructured":"Kuwana, T., Peterson, P.A. & Karlsson, L. Exit of major histocompatibility complex class II-invariant chain p35 complexes from the endoplasmic reticulum is modulated by phosphorylation. Proc. Natl. Acad. Sci. USA 95, 1056\u2013 1061 (1998).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnm0799745_CR35","doi-asserted-by":"publisher","first-page":"1227","DOI":"10.1096\/fasebj.11.14.9409541","volume":"11","author":"T Sommer","year":"1997","unstructured":"Sommer, T. & Wolf, D.H. Endoplasmic reticulum degradation: reverse protein flow of no return. FASEB J. 11, 1227\u20131233 (1997).","journal-title":"FASEB J."},{"key":"BFnm0799745_CR36","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/S0962-8924(97)01020-9","volume":"7","author":"JL Brodsky","year":"1997","unstructured":"Brodsky, J.L. & McCracken, A.A. ER-associated and proteasome-mediated protein degradation: how two topologically restricted events came together. Trends Cell Biol. 7, 151\u2013 156 (1997).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR37","doi-asserted-by":"publisher","first-page":"1883","DOI":"10.1083\/jcb.143.7.1883","volume":"143","author":"JA Johnston","year":"1998","unstructured":"Johnston, J.A., Ward, C.L. & Kopito, R.R. Aggresomes: a cellular response to misfolded proteins. J. Cell Biol. 143, 1883\u2013 1898 (1998).","journal-title":"J. Cell Biol."},{"key":"BFnm0799745_CR38","doi-asserted-by":"publisher","first-page":"S167","DOI":"10.1152\/physrev.1999.79.1.S167","volume":"79","author":"RR Kopito","year":"1999","unstructured":"Kopito, R.R. Biosynthesis and degradation of CFTR. Physiol. Rev. 79, S167\u2013173 (1999).","journal-title":"Physiol. Rev."},{"key":"BFnm0799745_CR39","doi-asserted-by":"publisher","first-page":"1075","DOI":"10.1126\/science.282.5391.1075","volume":"282","author":"J Hardy","year":"1998","unstructured":"Hardy, J. & Gwinn-Hardy, K. Genetic classification of primary neurodegenerative disease. Science 282, 1075\u20131079 (1998).","journal-title":"Science"},{"key":"BFnm0799745_CR40","doi-asserted-by":"publisher","first-page":"2611","DOI":"10.1091\/mbc.9.9.2611","volume":"9","author":"R Gardner","year":"1998","unstructured":"Gardner, R. et al. Sequence determinants for regulated degradation of yeast 3-hydroxy-3- methylglutaryl-CoA reductase, an integral endoplasmic reticulum membrane protein. Mol. Biol. Cell. 9, 2611\u20132626 (1998).","journal-title":"Mol. Biol. Cell."},{"key":"BFnm0799745_CR41","doi-asserted-by":"publisher","first-page":"12848","DOI":"10.1073\/pnas.95.22.12848","volume":"95","author":"A Nohturfft","year":"1998","unstructured":"Nohturfft, A., Brown, M.S. & Goldstein, J.L. Sterols regulate processing of carbohydrate chains of wild-type SREBP cleavage-activating protein (SCAP), but not sterol-resistant mutants Y298C or D443N. Proc. Natl. Acad. Sci. USA 95, 12848\u201312853 (1998).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnm0799745_CR42","doi-asserted-by":"publisher","first-page":"248","DOI":"10.1126\/science.280.5361.248","volume":"280","author":"HL Ploegh","year":"1998","unstructured":"Ploegh, H.L. Viral strategies of immune evasion. Science 280, 248\u2013253 (1998).","journal-title":"Science"},{"key":"BFnm0799745_CR43","first-page":"565","volume":"1","author":"F Margottin","year":"1998","unstructured":"Margottin, F. et al. A novel human WD protein, h-betaTrCP, that interacts with HIV-1 Vpu connects CD4 to the ER degradation pathway through an F-box motif. Cell 1, 565\u2013574 (1998).","journal-title":"Cell"},{"key":"BFnm0799745_CR44","doi-asserted-by":"publisher","first-page":"590","DOI":"10.1038\/25159","volume":"396","author":"A Yaron","year":"1998","unstructured":"Yaron, A. et al. Identification of the receptor component of the IkappaBalpha-ubiquitin ligase. Nature 396, 590-4 (1998).","journal-title":"Nature"},{"key":"BFnm0799745_CR45","doi-asserted-by":"crossref","first-page":"9054","DOI":"10.1128\/JVI.71.12.9054-9064.1997","volume":"71","author":"JR Doedens","year":"1997","unstructured":"Doedens, J.R., Giddings, J., T.H. & Kirkegaard, K. Inhibition of endoplasmic reticulum-to-Golgi traffic by poliovirus protein 3A: genetic and ultrastructural analysis. J. Virol. 71, 9054\u20139064 (1997).","journal-title":"J. Virol."},{"key":"BFnm0799745_CR46","doi-asserted-by":"publisher","first-page":"733","DOI":"10.1083\/jcb.140.4.733","volume":"140","author":"JM Lord","year":"1998","unstructured":"Lord, J.M. & Roberts, L.M. Toxin entry: retrograde transport through the secretory pathway. J. Cell Biol. 140, 733\u2013736 (1998).","journal-title":"J. Cell Biol."},{"key":"BFnm0799745_CR47","doi-asserted-by":"publisher","first-page":"20078","DOI":"10.1074\/jbc.270.34.20078","volume":"270","author":"JC Simpson","year":"1995","unstructured":"Simpson, J.C., Dascher, C., Roberts, L.M., Lord, J.M. & Balch, W.E. Ricin cytotoxicity is sensitive to recycling between the endoplasmic reticulum and the Golgi complex J. Biol. Chem. 270, 20078\u201320083 (1995).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR48","doi-asserted-by":"publisher","first-page":"510","DOI":"10.1038\/358510a0","volume":"358","author":"K Sandvig","year":"1992","unstructured":"Sandvig, K. et al. Retrograde transport of endocytosed Shiga toxin to the endoplasmic reticulum. Nature 358, 510\u2013 512 (1992).","journal-title":"Nature"},{"key":"BFnm0799745_CR49","doi-asserted-by":"publisher","first-page":"164","DOI":"10.1016\/S0968-0004(99)01386-9","volume":"24","author":"L Kuhn","year":"1999","unstructured":"Kuhn, L. Iron overload: molecular clues to its cause. Trends Biochem. Sci. 24, 164\u2013166 (1999).","journal-title":"Trends Biochem. Sci."},{"key":"BFnm0799745_CR50","doi-asserted-by":"publisher","first-page":"404","DOI":"10.1111\/j.1440-1746.1997.tb00451.x","volume":"12","author":"D Qu","year":"1997","unstructured":"Qu, D., Teckman, J.H. & Perlmutter, D.H. Alpha 1-antitrypsin deficiency associated liver disease. J. Gastroenterol. Hepatol. 12, 404\u2013 416 (1997).","journal-title":"J. Gastroenterol. Hepatol."},{"key":"BFnm0799745_CR51","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1055\/s-2008-1040399","volume":"12","author":"RN Sifers","year":"1992","unstructured":"Sifers, R.N., Finegold, M.J. & Woo, S.L.C. Molecular biology and genetics of alpha1-antitrypsin deficiency. Semin. Liver Dis. 12, 301\u2013 310 (1992).","journal-title":"Semin. Liver Dis."},{"key":"BFnm0799745_CR52","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1016\/S0014-5793(97)00423-7","volume":"409","author":"DA Brooks","year":"1997","unstructured":"Brooks, D.A. Protein processing: a role in the pathophysiology of genetic disease. FEBS Lett. 409, 115\u2013120 (1997).","journal-title":"FEBS Lett."},{"key":"BFnm0799745_CR53","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1177\/10454411980090040301","volume":"9","author":"MA Kukuruzinska","year":"1998","unstructured":"Kukuruzinska, M.A. & Lennon, K. Protein N-glycosylation: molecular genetics and functional significance. Crit Rev. Oral Biol. Med. 9, 415\u2013448 (1998).","journal-title":"Crit Rev. Oral Biol. Med."},{"key":"BFnm0799745_CR54","doi-asserted-by":"publisher","first-page":"1379","DOI":"10.1172\/JCI5731","volume":"103","author":"N Kalin","year":"1999","unstructured":"Kalin, N., Claabeta, A., Sommer, M., Puchelle, E. & Tummler, B. DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis. J. Clin. Invest. 103, 1379\u20131389 (1999).","journal-title":"J. Clin. Invest."},{"key":"BFnm0799745_CR55","doi-asserted-by":"publisher","first-page":"403","DOI":"10.1146\/annurev.bi.64.070195.002155","volume":"64","author":"DJ Prockop","year":"1995","unstructured":"Prockop, D.J. & Kivirikko, K.I. Collagens: molecular biology, diseases and potentials for therapy. Annu. Rev. Biochem. 64, 403\u2013434 (1995).","journal-title":"Annu. Rev. Biochem."},{"key":"BFnm0799745_CR56","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1159\/000111361","volume":"15","author":"M Hodes","year":"1993","unstructured":"Hodes, M., Pratt, V. & Dlouhy, S. Genetics of Perlizaeus-Merzbacher disease. Devel. Neurosci. 15, 383\u2013394 (1993).","journal-title":"Devel. Neurosci."},{"key":"BFnm0799745_CR57","doi-asserted-by":"publisher","first-page":"422","DOI":"10.1038\/ng0896-422","volume":"13","author":"A Gow","year":"1996","unstructured":"Gow, A. & Lazzarini, R.A. A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease. Nature Genet. 13, 422\u2013428 (1996).","journal-title":"Nature Genet."},{"key":"BFnm0799745_CR58","doi-asserted-by":"publisher","first-page":"1610","DOI":"10.1126\/science.280.5369.1610","volume":"280","author":"I Griffiths","year":"1998","unstructured":"Griffiths, I. et al. Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. Science 280, 1610\u20131613 (1998).","journal-title":"Science"},{"key":"BFnm0799745_CR59","first-page":"12","volume":"59","author":"ME Hodes","year":"1996","unstructured":"Hodes, M.E. & Dlouby, S.R. The proteolipid protein gene: double, double, ....and trouble. Am. J. Genet. 59, 12\u201315 (1996).","journal-title":"Am. J. Genet."},{"key":"BFnm0799745_CR60","first-page":"370","volume":"2","author":"P De Jonghe","year":"1997","unstructured":"De Jonghe, P., Timmerman, V., Nelis, E., Martin, J. & Van Broeckhoven, C. Charcot-Marie-Tooth disease and related peripheral neuropathies. J. Periph. Nervous Sys. 2, 370\u2013 387 (1997).","journal-title":"J. Periph. Nervous Sys."},{"key":"BFnm0799745_CR61","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1523\/JNEUROSCI.18-02-00731.1998","volume":"18","author":"D D'Urso","year":"1998","unstructured":"D'Urso, D., Prior, R., Greiner-Petter, R., Gabreels-Festen, A.A.W.M. & Muller, H.W. Overloaded endoplasmic reticulum-golgi compartments, a possible pathomechanism of peripheral neuropathies caused by mutations of the peripheral myelin protein PMP22. J. Neurosci. 18, 731\u2013740 (1998).","journal-title":"J. Neurosci."},{"key":"BFnm0799745_CR62","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1016\/S0092-8674(00)81163-0","volume":"93","author":"SB Pruisner","year":"1998","unstructured":"Pruisner, S.B., Scott, M.R., DeArmond, S.J. & Cohen, F.E. Prion protein biology. Cell 93, 337\u2013 348 (1998).","journal-title":"Cell"},{"key":"BFnm0799745_CR63","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/S0959-440X(98)80016-X","volume":"8","author":"JW Kelly","year":"1998","unstructured":"Kelly, J.W. The alternative conformations of amyloidogenic proteins and their multi-step assembly pathways. Curr. Opin. Struct. Biol. 8, 101\u2013106 (1998).","journal-title":"Curr. Opin. Struct. Biol."},{"key":"BFnm0799745_CR64","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1016\/S0962-8924(98)01363-4","volume":"8","author":"DJ Selkoe","year":"1998","unstructured":"Selkoe, D.J. The cell biology of beta-amyloid precursor protein and presenilin in Alzheimer's disease. Trends Cell Biol. 8, 447\u2013 453 (1998).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR65","doi-asserted-by":"publisher","first-page":"1021","DOI":"10.1038\/nm0997-1021","volume":"3","author":"DG Cook","year":"1997","unstructured":"Cook, D.G. et al. Alzheimer's ABeta(1-42) is generated in the endoplasmic reticulum\/intermediate compartment of NT2N cells. Nature Med. 3, 1021\u20131023 (1997).","journal-title":"Nature Med."},{"key":"BFnm0799745_CR66","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1046\/j.1471-4159.1998.70010001.x","volume":"70","author":"MP Mattson","year":"1998","unstructured":"Mattson, M.P., Guo, Q., Furukawa, K. & Pedersen, W.A. Presenilins, the endoplasmic reticulum and neuronal apoptosis in Alzheimer's disease. J. Neurochem. 70, 1\u201314 (1998).","journal-title":"J. Neurochem."},{"key":"BFnm0799745_CR67","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1038\/34910","volume":"391","author":"B De Strooper","year":"1998","unstructured":"De Strooper, B. et al. deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein. Nature 391, 387\u2013390 (1998).","journal-title":"Nature"},{"key":"BFnm0799745_CR68","doi-asserted-by":"publisher","first-page":"3590","DOI":"10.1074\/jbc.272.6.3590","volume":"272","author":"B De Strooper","year":"1997","unstructured":"De Strooper, B. et al. Phosphorylation, subcellular localization and membrane orientation of the Alzheimer's disease-associated presenilins. J. Biol. Chem. 272, 3590\u20133598 (1997).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR69","first-page":"719","volume":"49","author":"JD Culvenor","year":"1997","unstructured":"Culvenor, J.D. et al. Res. Alzheimer's disease associated presenilin 1 in neuronal cells: evidence for localization to the endoplasmic reticulum-Golgi intermediate compartment. J. Neurosci. 49, 719\u2013 731 (1997).","journal-title":"J. Neurosci."},{"key":"BFnm0799745_CR70","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1016\/S0962-8924(99)01576-7","volume":"9","author":"C Haass","year":"1999","unstructured":"Haass, C. & Mandelkow, E. Proteolysis by presenilins and the renaissance of tau. Trends Cell Biol. 9, 241\u2013244 (1999).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR71","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1038\/387288a0","volume":"387","author":"PC Wong","year":"1997","unstructured":"Wong, P.C. et al. Presenilin 1 is required for Notch1 and DII1 expression in the paraxial mesoderm. Nature 387, 288\u2013 292 (1997).","journal-title":"Nature"},{"key":"BFnm0799745_CR72","doi-asserted-by":"publisher","first-page":"770","DOI":"10.1126\/science.284.5415.770","volume":"284","author":"S Artavanis-Tsakonas","year":"1999","unstructured":"Artavanis-Tsakonas, S., Rand, M.D. & Lake, R.J. Notch signaling: cell fate control and signal integration in development. Science 284, 770\u2013 776 (1999).","journal-title":"Science"},{"key":"BFnm0799745_CR73","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1038\/19091","volume":"398","author":"G Struhl","year":"1999","unstructured":"Struhl, G. & Greenwald, I. Presenilin is required for activity and nuclear access of Notch in Drosophila. Nature 398, 522\u2013525 (1999).","journal-title":"Nature"},{"key":"BFnm0799745_CR74","doi-asserted-by":"publisher","first-page":"423","DOI":"10.1016\/S0092-8674(00)81583-4","volume":"94","author":"YM Chan","year":"1998","unstructured":"Chan, Y.M. & Jan, Y.N. Roles for proteolysis and trafficking in notch maturation and signal transduction. Cell 94, 423\u2013426 (1998).","journal-title":"Cell"},{"key":"BFnm0799745_CR75","doi-asserted-by":"publisher","first-page":"518","DOI":"10.1038\/19083","volume":"398","author":"B De Strooper","year":"1999","unstructured":"De Strooper, B. et al. A presenilin-1-dependent gamma-secretase-like protease mediates release of Notch intracellular domain. Nature 398, 518\u2013522 (1999).","journal-title":"Nature"},{"key":"BFnm0799745_CR76","doi-asserted-by":"publisher","first-page":"2029","DOI":"10.1091\/mbc.7.12.2029","volume":"7","author":"RY Hampton","year":"1996","unstructured":"Hampton, R.Y., Gardner, R.G. & Rine, J. Role of 26S proteasome and HRD genes in the degradation of 3-hydroxy-3-methylglutaryl-CoA reductase, an integral endoplasmic reticulum membrane protein. Mol. Biol. Cell 7, 2029\u20132044 (1996).","journal-title":"Mol. Biol. Cell"},{"key":"BFnm0799745_CR77","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1016\/S0962-8924(98)01368-3","volume":"8","author":"EM Mandelkow","year":"1998","unstructured":"Mandelkow, E.M. & Mandelkow, E. Tau in Alzheimer's disease. Trends Cell Biol. 8, 425\u2013 427 (1998).","journal-title":"Trends Cell Biol."},{"key":"BFnm0799745_CR78","doi-asserted-by":"publisher","first-page":"709","DOI":"10.1074\/jbc.272.2.709","volume":"272","author":"TW Loo","year":"1997","unstructured":"Loo, T.W. & Clarke, D.M. Correction of defective protein kinesis of human p-glycoprotein mutants by substrates and modulators. J. Biol. Chem. 272, 709\u2013712 (1997).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR79","doi-asserted-by":"publisher","first-page":"22796","DOI":"10.1074\/jbc.271.37.22796","volume":"271","author":"EG Mimnaugh","year":"1996","unstructured":"Mimnaugh, E.G., Chavany, C. & Neckers, L. Polyubiquitination and proteasomal degradation of the p185c-erbB-2 receptor protein-tyrosine kinase induced by geldanamycin. J. Biol. Chem. 271, 22796\u2013 22801 (1996).","journal-title":"J. Biol. Chem."},{"key":"BFnm0799745_CR80","unstructured":"Departments of Cell and Molecular Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037."}],"container-title":["Nature Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/nm0799_745.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nm0799_745","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/doifinder\/10.1038\/10466","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"http:\/\/www.nature.com\/articles\/nm0799_745.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T20:41:14Z","timestamp":1638391274000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/nm0799_745"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,7]]},"references-count":80,"journal-issue":{"issue":"7","published-print":{"date-parts":[[1999,7]]}},"alternative-id":["BFnm0799745"],"URL":"https:\/\/doi.org\/10.1038\/10466","relation":{},"ISSN":["1078-8956","1546-170X"],"issn-type":[{"value":"1078-8956","type":"print"},{"value":"1546-170X","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,7]]}}}