Original Manuscripts

Devaraneni, P., Conti, B., Matsumura, Y., Yang, Z., Johnson, A., Skach W. Insertion and Inversion of a Type II Signal Anchor Occurs in a Protected Environment of the Ribosome-Sec61 Complex. Cell. In Press 2011.

Khushoo A, Yang Z, Johnson A, Skach W.. Ligand-Driven Vectorial Folding of Ribosome-Bound Human CFTR NBD1. Molecular Cell. 41:682-92, 2011

Pratt, EB,. Tewson, P,. Bruederle CE, Skach W., Shyng SL. N-terminal transmembrane domain of SUR1 controls gating of Kir6.2 by modulating channel sensitivity to PIP2. J. Gen. Physiol. 2011, 137:299, 2011

Gubbens J, Kim SJ, Yang Z, Johnson AE, Skach W. In vitro incorporation of nonnatural amino acids into protein using tRNA(Cys)-derived opal, ochre, and amber suppressor tRNAs. RNA. 16:1660-1672, 2010

Hutt DM, Herman D, Rodrigues AP, Noel S, Pilewski JM, Matteson J, Hoch B, Kellner W, Kelly JW, Schmidt A, Thomas PJ, Matsumura Y, Skach W., Gentzsch M, Riordan JR, Sorscher EJ, Okiyoneda T, Yates JR 3rd, Lukacs GL, Frizzell RA, Manning G, Gottesfeld JM, Balch WE. Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nature Chem. Biol. 6:25-33, 2010

Yan FF, Pratt EB, Chen PC, Wang F, Skach W., David LL, Shyng SL. Role of Hsp90 in biogenesis of the beta-cell ATP-sensitive potassium channel complex. Mol. Biol. Cell. 21:1945-54, 2010

Pitonzo, D., Yang, Z., Matsumura, Y., Johnson, A., Skach, W. Sequence-Specific Retention and regulated integration of a nascent membrane protein by the endoplasmic reticulum Sec61 translocon. Mol. Biol. Cell, 20:685-698, 2009.

Crane, J., Van Hoek, A., Skach, W., Verkman, A. Aquaporin-4 dynamics in orthogonal arrays in live cells visualized by quantum dot single particle tracking. Mol. Biol. Cell. 19:3369-3378, 2008.

Daniel, C., Conti, B., Johnson, A., Skach, W. Control of translocation through the Sec61 translocon by nascent polypeptide structure within the ribosome. J. Biol. Chem. 283:20864-20873, 2008

Buck, T., Wagner, J., Grund, S., Skach, W. A novel tripartite structural motif involved in aquaporin topogenesis, monomer folding, and tetramerization. Nature Struct. Mol. Biol. 14:762-769, 2007

Wahlman, J., DeMartino, G., Skach, W., Bulleid, N., Brodsky, J., Johnson, A. Real-time fluorescence detection of ERAD substrate retrotranslocation in a mammalian in vitro system. Cell. 129:943-955, 2007

Shibatani, T., Carlson, E., Larabee, F., McCormack, A., Frueh, KJ., Skach, W. Global organization and function of mammalian cytosolic proteasome pools: Implications for PA28 and 19S regulatory complexes, Mol Biol Cell, 17:4962-4971, 2006.

Carlson, E., Pitonzo, D., Skach., W. p97 functions as an auxiliary factor to facilitate TM-domain extraction during CFTR ER-associated degradation. EMBO J. 25:4557-4566, 2006

Chia, D., Subbian, E., Buck, T., Hwa, V., Rosenfeld, R., Skach, W., Shinde, U., Rotwein, P. Aberrant folding of a mutant Stat5b causes growth hormone insensitivity and proteasomal dysfunction. J. Biol. Chem. 281:6552-6558, 2006

Oberdorf, J., Carlson, E., Skach, W. Uncoupling proteasome peptidase and ATPase activities results in cytosolic release of an ER polytopic protein. J. Cell. Sci. 119:303-313, 2006

Oberdorf, J., Pitonzo, D., Skach, W. An energy dependent maturation step required for release of the cystic fibrosis transmembrane conductance Regulator from early biosynthetic machinery. J. Biol. Chem., 280:38193-38202, 2005.

Sadlish, H. Pitonzo, D., Johnson, A., Skach, W. Sequential triage of transmembrane segments by Sec61α during biogenesis of a native multispanning membrane protein. Nature Struct. Mol. Biol., 12:970-878, 2005

Etchells, S., Meyer, A., Yam, A., Roobol, A., Miao, Y., Xhao, Y., Carden, M., Skach, W., Frydman, J., Johnson, A. The cotranslational contacts between ribosome-bound nascent polypeptides and the subunits of the hetero-oligomeric chaperonin TRiC probed by photocrosslinking, J. Biol. Chem. 280:28118-26, 2005.

Shibatani, T., David, L., McCormack, A., Frueh, K., Skach, W. Proteomic analysis of mammalian oligosaccharyltransferase reveals multiple subcomplexes that contain Sec61, TRAP and two potential new subunits. Biochem. 44:5982-5992, 2005.

Buck, T. Skach, W. Differential Stability of Biogenesis Intermediates Reveals a Common Pathway for Aquaporin-1 Topological Maturation, J. Biol. Chem. 280:2261-269, 2005.

Buck, T., Eledge, J., Skach, W. Evidence for stabilization of Aquaporin-2 folding mutants by N-linked glycosylation in the endoplasmic reticulum. Am. J. Physiol (Cell), 287:C1292-C1299, 2004.

Carveth, K., Buck, T., Anthony, V., Skach, W. Cooperativity and flexibility of CFTR transmembrane segments participate in membrane localizatin of a charged residue. J. Biol. Chem. In Press, 2002

Sidjanin, D., Parker-Wilson, D., Neuhaeuser-Klaus, A., Pretsch, W., Favor, J., Deen, P., Ohtaka-Maruyama, C., Lu, Y., Bragin, A., Skach, W., Chepelinsky, A., Grimes, P., Stambolian, D. A 76-bp deletion in the Mip gene causes autosomal dominant cataract in Hfi mice. Genomics, 74:313-319, 2001

Oberdorf, J., Carlson E., Skach, W. Redundancy of mammalian proteasome b-subunit function during endoplasmic reticulum associated degradation. Biochemistry, 40:13397-13405, 2001.

Foster, W., Helm, A., Turnbull, I., Gulati, H., Yang, B., Verkman, A., Skach, W. Identification of sequence determinants that direct alternate biogenesis pathways for aquaporin-1 and aquaporin-4. J. Biol. Chem. 275:34157-34165, 2000

Lu, Y., Turnbull, I., Bragin, A., K, Carveth, Verkman, A., Skach, W. Topologic reorientation of Aquaporin-1 during maturation in the endoplasmic reticulum. Mol. Biol Cell., 11:2973-2985, 2000

Kolosha, V., Anioa, E., deCespedes, C., Gitzelmann, R., Shih, L., Casco, T., Saborio. M., Trejos, R., Buist, N., Tedesco, T., Skach, W., Mitelmann, O., Leded, D., Huang, K., Stambolian, D. Novel mutations in 13 probands with galactokinase deficiency. Human Mutation, 15:447-453, 2000

Tu, L.-W., Wang, J., Helm, A., Skach, W., Deutsch, C. Transmembrane biogenesis of Kv1.3. Biochemistry, 39:824-836, 2000.

Xiong, X., Chong, E., Skach, W. Evidence that ER associated degradation of CFTR is linked to retrograde translocation from the ER membrane. J. Biol. Chem. 274:2616-2624, 1999

Jiang, Q., Mak, D., Devidas, S., Schweibert, E., Bragin, A., Zhang, Y., Skach, W., Guggino, W., Foskett, J.K., Engelhardt, J. CFTR associated ATP release is controlled by a "chloride sensor" within the channel pore. J. Cell. Biol. 143:645-658, 1998

Moss, K., Helm, A., Lu, Y., Bragin, A., Skach, W. Coupled translocation events generate topological heterogeneity at the endoplasmic reticulum. Mol. Biol. Cell. 9:2681-2697, 1998

Lu, Y., Xiong, X., Helm, A., Bragin, A., Kamani, K., Skach, W. Co- and Post-translational translocation mechanisms direct CFTR N-terminus transmembrane assembly. J. Biol. Chem. 273:568-576, 1998

Sheng, Z., Skach, W., Santarelli, V., Deutsch, C. Evidence for interaction between transmembrane segments in the assembly of Kv1.3. Biochemistry. 36:15501-15513, 1997

Xiong, X., Lu, Y., Bragin, A., Widdicombe, J.H., Cohn, J., Skach, W. Structural Cues involved in ER degradation of G85E and G91R mutant CFTR. J. Clin. Invest. 100:1079-1088, 1997

Bargsma, D., Ai, Y., Skach, W., Nesburn, K., Anoia, E., Van Horn, S., Stambolian, D. Fine structure of the human galactoskinase GALK1 gene. Genome Research, 6:980-985, 1996

Tu, L.-W., Santarelli, V., Sheng, Z.-F., Skach, W., Pain, D., Deutsch, C. Voltage-Gated K+ channels contain multiple intersubunit Association Sites. J. Biol. Chem. 271:18904-18911, 1996

Shi, L.-B., Skach, W., Ma, T., Verkman, A. Distinct biogenesis mechanisms for the water channels MIWC and CHIP28 at the endoplasmic reticulum. Biochemistry 34:8250-8256, 1995.

Ohrui, T., Skach, W., Thompson, M., Matsumoto-Pon, J., Calayag, M.C., Widdicombe, J. Radiotracer studies of CFTR expressed in Xenopus oocytes. Am J. Physiol. (Cell Phys.) 266(6 Pt 1):C1586-93, 1994

Skach, W., and Lingappa, V. Transmembrane orientation and topogenesis of the 3rd and 4th membrane -spanning regions of human P-glycoprotein (MDR1). Ca. Res., 54:3202-3209, 1994

Shi, L.-B., Skach, W., Verkman, A.S. Functional independence of monomeric CHIP28 water channels revealed by expression of wild-type-mutant heterodimers. J. Biol. Chem. 269:10417-10422, 1994

Skach, W., Shi, L.-B., Calayag, M.C., Lingappa, V., Verkman, A.S. Biogenesis and transmembrane topology of the human CHIP28 water channel in the endoplasmic reticulum. J. Cell Biol., 125:803-815, 1994

Hasegawa, H., Ma, T., Skach, W., Matthay, M., Verkman, A.S. Molecular cloning of a mercurial-insensitive water channel (MIWC30) expressed in selected water transporting epithelia. J. Biol. Chem., 269:5497-5500, 1994

Ma, T., Hasegawa, H., Skach, W., Frigeri, A., Verkman, A. Expression, functional analysis and in situ hybridization of a cloned rat kidney collecting duct water channel. Am. J. Physiol. (Cell Phys.), 266:C189-C197, 1994

Ma, T., Frigeri, A., Skach, W., Verkman, A.S. Cloning of a novel rat kidney cDNA homologous to CHIP28 and WCH-CD water channels. Biochem. Biophys. Res. Comm., 197:654-659, 1993

Skach, W., Lingappa, V. Amino terminus assembly of human P-glycoprotein at the endoplasmic reticulum is directed by cooperative actions of internal sequences. J. Biol. Chem. 268:23552-23561, 1993

Shen, B-Q., Barthelson, R., Skach, W., Gruenert, D., Sigal, E., Mrsny, R., Widdicombe, J. Mechanism of Inhibition of cAMP-dependent epithelial Cl secretion by phorbol esters. J. Biol. Chem. 268:19070-19075, 1993

Zhang, R., Skach, W., Hasegawa, H., van Hoek, A., Verkman, A. Cloning, functional analysis and cell localization of a kidney proximal tubule water transporter homologous to CHIP28. J Cell Biol. 120:359-369, 1993

Skach, W., Calayag, M.C., Lingappa, V. Evidence for an alternate model of human P-glycoprotein structure and biogenesis. J. Biol. Chem. 268:6903-6908, 1993

Hasegawa, H., Skach, W., Baker, O. Calayag, M.C., Lingappa, V., Verkman, A.S. A multifunctional aqueous channel formed by CFTR. Science. 258:1477-1479, 1992

Editorials, Reviews, and Chapters

Brodsky, J., Skach, W. Protein Folding and Quality Control in the Endoplasmic Reticulum: Recent Lessons from Yeast and Mammalian Cell Systems. Current Opinion in Cell Biology. In Press, 2011,

Matsumura, Y., Rooney, L-A., Skach, W. In vitro methods for CFTR biogenesis. In Press, 2011

Balch, W., Braakman, I., Brodsky, J., Frizzell, R., Guggino, W., Lukacs, G., Penland, C., Pollard, H., Skach, W., Sorscher, E., Thomas, P. Folding biology of cystic fibrosis: A consortium-based approach to disease. In Protein Misfolding Diseases: Current and emerging principles and therapies. eds Ramirez-Alvarado, M., Kelly, J., Dobson, C., Wiley, 425-452, 2010

Matsumura, Y., Skach, W. Protein export from the endoplasmic reticulum to cytosol: In vitro methods. Encyclopedia of Life Sciences (ELS), 2009 DOI:10.1002/9780470015902.a0003430.pub2

Skach, W. Cellular mechanisms of membrane protein folding. Nature Struct. Mol. Biol. 16:606-612, 2009 PMCID: PMC2814870

Skach, W. Cellular Mechanisms of Membrane Protein Folding, Nature Struct. Mol. Biol. 2009.

Skach, W. The expanding role of the ER translocon in membrane protein folding. J. Cell. Biol. 1179:333-1335, 2007

Skach, W. Pharmacological chaperoning: Two 'hits' are better than one. Biochem. J. 406:e1-2, 2007.

Skach, W. CFTR: New members join the fold. Cell. 127:673-675, 2006.

Pitonzo, D., Skach W. Molecular mechanisms of aquaporin biogenesis by the endoplasmic reticulum Sec61 translocon. Bioch. Biophys. Acta. 1758:976-988, 2006.

Carlson, E., Bays, N., David, L., Skach, W. Reticulocyte lysate as a model system to study ER membrane protein degradation. Methods in Molecular Biology: Ubiquitin proteasome protocol, eds, Patterson, C and Cyr, D., The Humana Press Inc., Towata, NJ. 301:185-205, 2005.

Sadlish, H., Skach, W. Biogenesis of CFTR and other polytopic membrane proteins: new roles for the ribosome-translocon complex. Journal of Membrane Biology, 202:115-126, 2004.

Anthony, V., Skach W. Molecular mechanism of P-glycoprotein assembly into cellular membranes. Current Protein and Peptide Science, 3:485-501, 2002.

Oberdorf, J., and Skach, W. In vitro reconstitution of CFTR biogenesis and degradation. in Methods in Molecular Medicine, vol. 70. Cystic Fibrosis: Methods and Protocols. ed. W. Skach. The Humana Press Inc., Towata, NJ. pp. 295-310, 2002.

Verkman ,A., Yang, B., Skach, W., Mitra, A, Song, Y., Manley, G., Ma, T. Genetic and biophysical approaches to study water channel biology. in Current Topics in Membranes: Water Channels. (eds. S. Hohmann, P. Agre and S. Nielsen), Academic Press. 51:185-233, 2001.

Turnbull, I., Lu, Y., Verkman, A., Skach, W. Biogenesis and folding of aquaporin water channels in the endoplasmic reticulum. in Molecular Biology and Physiology of Water and Solute Transport: Fundamental and Applied Aspects. (ed. S. Hohmann) Kluwer Academic/Plenum Publishers. pp. 49-55, 2000.

Skach, W. Defects in Processing and Trafficking of CFTR. Kidney International, 57:825-831, 2000.

Skach, W. The Topology of P-glycoproteins. in ABC Transporters: Biochemical, Cellular and Molecular Aspects, Methods in Enzymology, (eds. S. Ambudkar and M. Gottesman), 292:265-279, 1998.

Verkman, A.S., van Hoek, A., Ma, T., Frigeri, A., Skach, W., Mitra, A., Tamarappoo, B., Farinas, J. Water transport across mammalian cell membranes. Am. J. Physiol. 270 (Cell Physiol. 39) C12-C30, 1996.

Verkman, A.S., Shi, L.B., Frigeri, A., Hasegawa, H., Farinas, J., Mitra, A., Skach, W., Brown, D., van Hoek, A., Ma, T. Structure and function of kidney water channels. Kidney International. 48:1069-1081, 1995.

Skach, W., Lingappa, V. Intracellular trafficking of pre-(pro-)proteins across RER membranes. in Mechanisms of Intracellular Trafficking and Processing of Pro-proteins. Loh, Y. (ed), CRC Press inc. pp 19-77, 1993.

Skach, W., Fitz, J. Common Medical Ward Problems. in Handbook of Medical Treatment; 18th edition, pp 1-21. Jones Medical Publications, 1988.

Skach, W., Cadman, E. Cancer Chemotherapy and Oncological Emergencies. in Handbook of Medical Treatment , 18th edition, pp 214-231. Jones Medical Publications 1988.

Books

Skach, W., Daley, C., Forsmark, C., (eds) Handbook of Medical Treatment ; 18th Edition, Jones Medical Publications, 1988

Skach, W. (ed.) Cystic Fibrosis: Methods and Protocols, in series Methods in Molecular Medicine: The Humana Press Inc., Towota, NJ., 2002