肌間線蛋白是一分子量53 kDa，表現在肌肉的中間絲蛋白，它形成外肌節的細胞骨架，將肌纖維串連起來且把收縮裝置連接至粒線體、細胞核和細胞間接合。當肌間線蛋白突變將導致肌間線蛋白肌病變，這是一種會影響骨骼肌和心肌的嚴重罕見疾病。肌間線蛋白肌病變的特徵是肌節間的大量含肌間線蛋白粒絲狀蛋白堆積物。且經由先前的發現，一小型熱休克蛋白B-水晶體的突變亦可造成與肌間線蛋白肌病變相同的病理特徵，支持B-水晶體和肌間線蛋白功能性上交互作用的首次證據。在本次研究中，我們使用許多生化方法驗證B-水晶體與肌間線蛋白的交互作用關係。此外，我們也設計出可專一性辨認被凋亡蛋白酶6處理的N端肌間線蛋白片段的抗體。當細胞表現疾病特有的肌間線蛋白突變時，透過這種抗體也可偵測到N端肌間線蛋白的產生。在體外環境中，N端肌間線蛋白無法自行產生十奈米寬的中間絲構造，反而形成高密度的蛋白堆積物。Co-assembly的研究也顯示N端肌間線蛋白會干擾正常肌間線蛋白的結合，促使其形成蛋白堆積物。綜合以上研究結果，可知藉由凋亡蛋白酶處理所產生的N端肌間線蛋白有傾向形成蛋白堆積物的能力，且建立了N端肌間線蛋白促進細胞凋亡能力的基礎，以及藉由影響粒線體功能以致危害細胞存活，這都是肌間線蛋白肌病變的重要病理生理學特徵。

Desmin is a 53 kDa muscle-specific intermediate filament protein, which forms extrasarcomeric cytoskeleton that interconnects myofibrils and links the contractile apparatus to mitochondria, nuclei and intercellular junctions. Mutations in desmin cause desmin-related myopathy (DRM), a rare and devastating genetic disorder affecting both skeletal and cardiac muscles. DRM is characterized by intrasarcolemmal aggregates of granulofilamentous material containing predominantly desmin. Since that initial discovery, mutations in the small heat shock protein B-crystallin was found to phenocopy DRM caused by desmin mutation, providing the first genetic evidence in support of a functional interaction of B-crystallin and desmin. In this study, we used a variety of biochemical assay to show that B-crystallin can interact with desmin. In addition, we generated polyclonal antibodies specific to the N-terminal fragment of desmin (N-desmin) generated by caspase 6 cleavage. Using this antibody, N-desmin was detected in cells undergoing apoptosis. In vitro assembly studies had demonstrated that the N-desmin was unable to form 10-nm intermediate filaments, but instead form electron-dense aggregates. Coassembly studies showed that N-desmin interfered with the normal assembly of intact desmin in a way that promoted filament aggregation. Taken together, these data suggest caspase-mediated cleavage of desmin produces N-terminal proteolytic fragment that is prone to aggregation. Furthermore, N-desmin and other disease-causing mutations interfere intermediate filaments networks and mitochondrial phenotypes in cells. This study provides a firm and testable foundation to the hypothesis that the N-terminal desmin is pro-apoptotic, affecting mitochondria function that compromises cell viability as key part of the pathophysiology of DRM.

Almahbobi, G., L. J. Williams and P. F. Hall (1992). "Attachment of mitochondria to intermediate filaments in adrenal cells: relevance to the regulation of steroid synthesis." Exp Cell Res 200(2): 361-369.
Arrigo, A. P., J. P. Suhan and W. J. Welch (1988). "Dynamic changes in the structure and intracellular locale of the mammalian low-molecular-weight heat shock protein." Mol Cell Biol 8(12): 5059-5071.
Bar, H., B. Goudeau, S. Walde, M. Casteras-Simon, N. Mucke, A. Shatunov, Y. P. Goldberg, C. Clarke, J. L. Holton, B. Eymard, H. A. Katus, M. Fardeau, L. Goldfarb, P. Vicart and H. Herrmann (2007). "Conspicuous involvement of desmin tail mutations in diverse cardiac and skeletal myopathies." Hum Mutat 28(4): 374-386.
Bar, H., A. Kostareva, G. Sjoberg, T. Sejersen, H. A. Katus and H. Herrmann (2006). "Forced expression of desmin and desmin mutants in cultured cells: impact of myopathic missense mutations in the central coiled-coil domain on network formation." Exp Cell Res 312(9): 1554-1565.
Bar, H., N. Mucke, H. A. Katus, U. Aebi and H. Herrmann (2007). "Assembly defects of desmin disease mutants carrying deletions in the alpha-helical rod domain are rescued by wild type protein." J Struct Biol 158(1): 107-115.
Bar, H., N. Mucke, A. Kostareva, G. Sjoberg, U. Aebi and H. Herrmann (2005). "Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages." Proc Natl Acad Sci U S A 102(42): 15099-15104.
Bar, H., N. Mucke, P. Ringler, S. A. Muller, L. Kreplak, H. A. Katus, U. Aebi and H. Herrmann (2006). "Impact of disease mutations on the desmin filament assembly process." J Mol Biol 360(5): 1031-1042.
Bar, H., S. V. Strelkov, G. Sjoberg, U. Aebi and H. Herrmann (2004). "The biology of desmin filaments: how do mutations affect their structure, assembly, and organisation?" J Struct Biol 148(2): 137-152.
Behlke, J., G. Lutsch, M. Gaestel and H. Bielka (1991). "Supramolecular structure of the recombinant murine small heat shock protein hsp25." FEBS Lett 288(1-2): 119-122.
Bova, M. P., O. Yaron, Q. Huang, L. Ding, D. A. Haley, P. L. Stewart and J. Horwitz (1999). "Mutation R120G in alphaB-crystallin, which is linked to a desmin-related myopathy, results in an irregular structure and defective chaperone-like function." Proc Natl Acad Sci U S A 96(11): 6137-6142.
Brakenhoff, R. H., A. H. Guerts van Kessel, M. Oldenburg, J. T. Wijnen, H. Bloemendal, P. Meera Khan and J. G. Schoenmakers (1990). "Human alpha B-crystallin (CRYA2) gene mapped to chromosome 11q12-q23." Hum Genet 85(2): 237-240.
Byun, Y., F. Chen, R. Chang, M. Trivedi, K. J. Green and V. L. Cryns (2001). "Caspase cleavage of vimentin disrupts intermediate filaments and promotes apoptosis." Cell Death Differ 8(5): 443-450.
Carmo-Fonseca, M. and J. F. David-Ferreira (1990). "Interactions of intermediate filaments with cell structures." Electron Microsc Rev 3(1): 115-141.
Caulin, C., G. S. Salvesen and R. G. Oshima (1997). "Caspase cleavage of keratin 18 and reorganization of intermediate filaments during epithelial cell apoptosis." J Cell Biol 138(6): 1379-1394.
Chen, F., R. Chang, M. Trivedi, Y. Capetanaki and V. L. Cryns (2003). "Caspase proteolysis of desmin produces a dominant-negative inhibitor of intermediate filaments and promotes apoptosis." J Biol Chem 278(9): 6848-6853.
Clark, A. R., C. E. Naylor, C. Bagneris, N. H. Keep and C. Slingsby (2011). "Crystal structure of R120G disease mutant of human alphaB-crystallin domain dimer shows closure of a groove." J Mol Biol 408(1): 118-134.
Clark, K. A., A. S. McElhinny, M. C. Beckerle and C. C. Gregorio (2002). "Striated muscle cytoarchitecture: an intricate web of form and function." Annu Rev Cell Dev Biol 18: 637-706.
Clemen, C. S., H. Herrmann, S. V. Strelkov and R. Schroder (2013). "Desminopathies: pathology and mechanisms." Acta Neuropathol 125(1): 47-75.
Dagvadorj, A., B. Goudeau, D. Hilton-Jones, J. K. Blancato, A. Shatunov, M. Simon-Casteras, W. Squier, J. W. Nagle, L. G. Goldfarb and P. Vicart (2003). "Respiratory insufficiency in desminopathy patients caused by introduction of proline residues in desmin c-terminal alpha-helical segment." Muscle Nerve 27(6): 669-675.
David-Ferreira, K. L. and J. F. David-Ferreira (1980). "Association between intermediate-sized filaments and mitochondria in rat Leydig cells." Cell Biol Int Rep 4(7): 655-662.
Der Perng, M., M. Su, S. F. Wen, R. Li, T. Gibbon, A. R. Prescott, M. Brenner and R. A. Quinlan (2006). "The Alexander disease-causing glial fibrillary acidic protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alpha B-crystallin and HSP27." Am J Hum Genet 79(2): 197-213.
Djabali, K., B. de Nechaud, F. Landon and M. M. Portier (1997). "AlphaB-crystallin interacts with intermediate filaments in response to stress." J Cell Sci 110(21): 2759-2769.
Dubin, R. A., A. H. Ally, S. Chung and J. Piatigorsky (1990). "Human alpha B-crystallin gene and preferential promoter function in lens." Genomics 7(4): 594-601.
Fardeau, M., J. Godet-Guillain, F. M. Tome, H. Collin, S. Gaudeau, C. Boffety and P. Vernant (1978). "A new familial muscular disorder demonstrated by the intra-sarcoplasmic accumulation of a granulo-filamentous material which is dense on electron microscopy." Rev Neurol (Paris) 134(6-7): 411-425.
Fardeau, M., P. Vicart, A. Caron, D. Chateau, M. Chevallay, H. Collin, F. Chapon, D. Duboc, B. Eymard, F. M. Tome, J. M. Dupret, D. Paulin and P. Guicheney (2000). "[Familial myopathy with desmin storage seen as a granulo-filamentar, electron-dense material with mutation of the alphaB-cristallin gene]." Rev Neurol (Paris) 156(5): 497-504.
Fuchs, E. and K. Weber (1994). "Intermediate filaments: structure, dynamics, function, and disease." Annu Rev Biochem 63: 345-382.
Furst, D. O., M. Osborn and K. Weber (1989). "Myogenesis in the mouse embryo: differential onset of expression of myogenic proteins and the involvement of titin in myofibril assembly." J Cell Biol 109(2): 517-527.
Goebel, H. H. (1995). "Desmin-related neuromuscular disorders." Muscle Nerve 18(11): 1306-1320.
Goebel, H. H., T. Voit, I. Warlo, K. Jacobs, U. Johannsen and C. R. Muller (1994). "Immunohistologic and electron microscopic abnormalities of desmin and dystrophin in familial cardiomyopathy and myopathy." Rev Neurol (Paris) 150(6-7): 452-459.
Goldfarb, L. G., K. Y. Park, L. Cervenakova, S. Gorokhova, H. S. Lee, O. Vasconcelos, J. W. Nagle, C. Semino-Mora, K. Sivakumar and M. C. Dalakas (1998). "Missense mutations in desmin associated with familial cardiac and skeletal myopathy." Nat Genet 19(4): 402-403.
Goldfarb, L. G., P. Vicart, H. H. Goebel and M. C. Dalakas (2004). "Desmin myopathy." Brain 127(4): 723-734.
Goudeau, B., A. Dagvadorj, F. Rodrigues-Lima, P. Nedellec, M. Casteras-Simon, E. Perret, S. Langlois, L. Goldfarb and P. Vicart (2001). "Structural and functional analysis of a new desmin variant causing desmin-related myopathy." Hum Mutat 18(5): 388-396.
Hengartner, M. O. (2000). "The biochemistry of apoptosis." Nature 407(6805): 770-776.
Herrmann, H. and U. Aebi (1998). "Structure, assembly, and dynamics of intermediate filaments." Subcell Biochem 31: 319-362.
Herrmann, H., H. Bar, L. Kreplak, S. V. Strelkov and U. Aebi (2007). "Intermediate filaments: from cell architecture to nanomechanics." Nat Rev Mol Cell Biol 8(7): 562-573.
Herrmann, H., M. Haner, M. Brettel, N. O. Ku and U. Aebi (1999). "Characterization of distinct early assembly units of different intermediate filament proteins." J Mol Biol 286(5): 1403-1420.
Herrmann, H., S. V. Strelkov, P. Burkhard and U. Aebi (2009). "Intermediate filaments: primary determinants of cell architecture and plasticity." J Clin Invest 119(7): 1772-1783.
Hijikata, T., T. Murakami, M. Imamura, N. Fujimaki and H. Ishikawa (1999). "Plectin is a linker of intermediate filaments to Z-discs in skeletal muscle fibers." J Cell Sci 112(6): 867-876.
Houck, S. A., A. Landsbury, J. I. Clark and R. A. Quinlan (2011). "Multiple sites in alphaB-crystallin modulate its interactions with desmin filaments assembled in vitro." PLoS One 6(11): e25859.
Iwaki, A., T. Iwaki, J. E. Goldman and R. K. Liem (1990). "Multiple mRNAs of rat brain alpha-crystallin B chain result from alternative transcriptional initiation." J Biol Chem 265(36): 22197-22203.
Jakob, U., M. Gaestel, K. Engel and J. Buchner (1993). "Small heat shock proteins are molecular chaperones." J Biol Chem 268(3): 1517-1520.
Jimenez-Mallebrera, C., S. C. Brown, C. A. Sewry and F. Muntoni (2005). "Congenital muscular dystrophy: molecular and cellular aspects." Cell Mol Life Sci 62(7-8): 809-823.
Kay, L., Z. Li, M. Mericskay, J. Olivares, L. Tranqui, E. Fontaine, T. Tiivel, P. Sikk, T. Kaambre, J. L. Samuel, L. Rappaport, Y. Usson, X. Leverve, D. Paulin and V. A. Saks (1997). "Study of regulation of mitochondrial respiration in vivo. An analysis of influence of ADP diffusion and possible role of cytoskeleton." Biochim Biophys Acta 1322(1): 41-59.
Klauke, B., S. Kossmann, A. Gaertner, K. Brand, I. Stork, A. Brodehl, M. Dieding, V. Walhorn, D. Anselmetti, D. Gerdes, B. Bohms, U. Schulz, E. Zu Knyphausen, M. Vorgerd, J. Gummert and H. Milting (2010). "De novo desmin-mutation N116S is associated with arrhythmogenic right ventricular cardiomyopathy." Hum Mol Genet 19(23): 4595-4607.
Kostareva, A., G. Sjoberg, J. Bruton, S. J. Zhang, J. Balogh, A. Gudkova, B. Hedberg, L. Edstrom, H. Westerblad and T. Sejersen (2008). "Mice expressing L345P mutant desmin exhibit morphological and functional changes of skeletal and cardiac mitochondria." J Muscle Res Cell Motil 29(1): 25-36.
Krammer, P. H. (1999). "CD95(APO-1/Fas)-mediated apoptosis: live and let die." Adv Immunol 71: 163-210.
Lazarides, E. (1980). "Intermediate filaments as mechanical integrators of cellular space." Nature 283(5744): 249-256.
Leterrier, J. F., D. A. Rusakov, B. D. Nelson and M. Linden (1994). "Interactions between brain mitochondria and cytoskeleton: evidence for specialized outer membrane domains involved in the association of cytoskeleton-associated proteins to mitochondria in situ and in vitro." Microsc Res Tech 27(3): 233-261.
Li, Z., P. Marchand, J. Humbert, C. Babinet and D. Paulin (1993). "Desmin sequence elements regulating skeletal muscle-specific expression in transgenic mice." Development 117(3): 947-959.
Li, Z., M. Mericskay, O. Agbulut, G. Butler-Browne, L. Carlsson, L. E. Thornell, C. Babinet and D. Paulin (1997). "Desmin is essential for the tensile strength and integrity of myofibrils but not for myogenic commitment, differentiation, and fusion of skeletal muscle." J Cell Biol 139(1): 129-144.
Li, Z. L., A. Lilienbaum, G. Butler-Browne and D. Paulin (1989). "Human desmin-coding gene: complete nucleotide sequence, characterization and regulation of expression during myogenesis and development." Gene 78(2): 243-254.
Lin, A., G. Krockmalnic and S. Penman (1990). "Imaging cytoskeleton--mitochondrial membrane attachments by embedment-free electron microscopy of saponin-extracted cells." Proc Natl Acad Sci U S A 87(21): 8565-8569.
Linden, M., Z. Li, D. Paulin, T. Gotow and J. F. Leterrier (2001). "Effects of desmin gene knockout on mice heart mitochondria." J Bioenerg Biomembr 33(4): 333-341.
MacArthur, M. W. and J. M. Thornton (1991). "Influence of proline residues on protein conformation." J Mol Biol 218(2): 397-412.
MacFarlane, M., W. Merrison, D. Dinsdale and G. M. Cohen (2000). "Active caspases and cleaved cytokeratins are sequestered into cytoplasmic inclusions in TRAIL-induced apoptosis." J Cell Biol 148(6): 1239-1254.
Maloyan, A., A. Sanbe, H. Osinska, M. Westfall, D. Robinson, K. Imahashi, E. Murphy and J. Robbins (2005). "Mitochondrial dysfunction and apoptosis underlie the pathogenic process in alpha-B-crystallin desmin-related cardiomyopathy." Circulation 112(22): 3451-3461.
Milner, D. J., M. Mavroidis, N. Weisleder and Y. Capetanaki (2000). "Desmin cytoskeleton linked to muscle mitochondrial distribution and respiratory function." J Cell Biol 150(6): 1283-1298.
Mose-Larsen, P., R. Bravo, S. J. Fey, J. V. Small and J. E. Celis (1982). "Putative association of mitochondria with a subpopulation of intermediate-sized filaments in cultured human skin fibroblasts." Cell 31(3): 681-692.
Muchowski, P. J., M. M. Valdez and J. I. Clark (1999). "AlphaB-crystallin selectively targets intermediate filament proteins during thermal stress." Invest Ophthalmol Vis Sci 40(5): 951-958.
Munoz-Marmol, A. M., G. Strasser, M. Isamat, P. A. Coulombe, Y. Yang, X. Roca, E. Vela, J. L. Mate, J. Coll, M. T. Fernandez-Figueras, J. J. Navas-Palacios, A. Ariza and E. Fuchs (1998). "A dysfunctional desmin mutation in a patient with severe generalized myopathy." Proc Natl Acad Sci U S A 95(19): 11312-11317.
Nicholl, I. D. and R. A. Quinlan (1994). "Chaperone activity of alpha-crystallins modulates intermediate filament assembly." EMBO J 13(4): 945-953.
Oshima, R. G. (2002). "Apoptosis and keratin intermediate filaments." Cell Death Differ 9(5): 486-492.
Park, K. Y., M. C. Dalakas, C. Semino-Mora, H. S. Lee, S. Litvak, K. Takeda, V. J. Ferrans and L. G. Goldfarb (2000). "Sporadic cardiac and skeletal myopathy caused by a de novo desmin mutation." Clin Genet 57(6): 423-429.
Paulin, D. and Z. Li (2004). "Desmin: a major intermediate filament protein essential for the structural integrity and function of muscle." Exp Cell Res 301(1): 1-7.
Penisson-Besnier, I., C. Dumez, D. Chateau, F. Dubas and M. Fardeau (1998). "Autosomal dominant late adult onset distal leg myopathy." Neuromuscul Disord 8(7): 459-466.
Perng, M. D., L. Cairns, I. P. van den, A. Prescott, A. M. Hutcheson and R. A. Quinlan (1999). "Intermediate filament interactions can be altered by HSP27 and alphaB-crystallin." J Cell Sci 112 (13): 2099-2112.
Perng, M. D., S. F. Wen, I. P. van den, A. R. Prescott and R. A. Quinlan (2004). "Desmin aggregate formation by R120G alphaB-crystallin is caused by altered filament interactions and is dependent upon network status in cells." Mol Biol Cell 15(5): 2335-2346.
Petty, R. K., A. E. Harding and J. A. Morgan-Hughes (1986). "The clinical features of mitochondrial myopathy." Brain 109 (5): 915-938.
Price, M. G. (1984). "Molecular analysis of intermediate filament cytoskeleton--a putative load-bearing structure." Am J Physiol 246(4 Pt 2): H566-572.
Raats, J. M., G. Schaart, J. B. Henderik, A. van der Kemp, I. Dunia, E. L. Benedetti, F. R. Pieper, F. C. Ramaekers and H. Bloemendal (1996). "Muscle-specific expression of a dominant negative desmin mutant in transgenic mice." Eur J Cell Biol 71(3): 221-236.
Reipert, S., F. Steinbock, I. Fischer, R. E. Bittner, A. Zeold and G. Wiche (1999). "Association of mitochondria with plectin and desmin intermediate filaments in striated muscle." Exp Cell Res 252(2): 479-491.
Schaart, G., C. Viebahn, W. Langmann and F. Ramaekers (1989). "Desmin and titin expression in early postimplantation mouse embryos." Development 107(3): 585-596.
Schroder, R., B. Goudeau, M. C. Simon, D. Fischer, T. Eggermann, C. S. Clemen, Z. Li, J. Reimann, Z. Xue, S. Rudnik-Schoneborn, K. Zerres, P. F. van der Ven, D. O. Furst, W. S. Kunz and P. Vicart (2003). "On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria." Hum Mol Genet 12(6): 657-669.
Schroder, R. and B. Schoser (2009). "Myofibrillar myopathies: a clinical and myopathological guide." Brain Pathol 19(3): 483-492.
Schultheiss, T., Z. X. Lin, H. Ishikawa, I. Zamir, C. J. Stoeckert and H. Holtzer (1991). "Desmin/vimentin intermediate filaments are dispensable for many aspects of myogenesis." J Cell Biol 114(5): 953-966.
Schutte, B., M. E. Henfling, F. K. Verheyen, G. Li, G. V. Tolstonog and F. C. Ramaekers (2009). "The caspase-9 derived C-terminal fragment of cytokeratin 18 modulates topoisomerase action." Int J Oncol 35(3): 625-630.
Selcen, D. (2011). "Myofibrillar myopathies." Neuromuscul Disord 21(3): 161-171.
Song, S., M. J. Hanson, B. F. Liu, L. T. Chylack and J. J. Liang (2008). "Protein-protein interactions between lens vimentin and alphaB-crystallin using FRET acceptor photobleaching." Mol Vis 14: 1282-1287.
Sugawara, M., K. Kato, M. Komatsu, C. Wada, K. Kawamura, P. S. Shindo, P. N. Yoshioka, K. Tanaka, S. Watanabe and I. Toyoshima (2000). "A novel de novo mutation in the desmin gene causes desmin myopathy with toxic aggregates." Neurology 55(7): 986-990.
Tan, E., H. Topaloglu, C. Sewry, Y. Zorlu, I. Naom, S. Erdem, M. D'Alessandro, F. Muntoni and V. Dubowitz (1997). "Late onset muscular dystrophy with cerebral white matter changes due to partial merosin deficiency." Neuromuscul Disord 7(2): 85-89.
Taylor, M. R., D. Slavov, L. Ku, A. Di Lenarda, G. Sinagra, E. Carniel, K. Haubold, M. M. Boucek, D. Ferguson, S. L. Graw, X. Zhu, J. Cavanaugh, C. C. Sucharov, C. S. Long, M. R. Bristow, P. Lavori, L. Mestroni, R. Familial Cardiomyopathy and B. D. Bank (2007). "Prevalence of desmin mutations in dilated cardiomyopathy." Circulation 115(10): 1244-1251.
Taylor, R. P. and I. J. Benjamin (2005). "Small heat shock proteins: a new classification scheme in mammals." J Mol Cell Cardiol 38(3): 433-444.
Thornell, L., L. Carlsson, Z. Li, M. Mericskay and D. Paulin (1997). "Null mutation in the desmin gene gives rise to a cardiomyopathy." J Mol Cell Cardiol 29(8): 2107-2124.
Vendelin, M., N. Beraud, K. Guerrero, T. Andrienko, A. V. Kuznetsov, J. Olivares, L. Kay and V. A. Saks (2005). "Mitochondrial regular arrangement in muscle cells: a "crystal-like" pattern." Am J Physiol Cell Physiol 288(3): C757-767.
Vicart, P., A. Caron, P. Guicheney, Z. Li, M. C. Prevost, A. Faure, D. Chateau, F. Chapon, F. Tome, J. M. Dupret, D. Paulin and M. Fardeau (1998). "A missense mutation in the alphaB-crystallin chaperone gene causes a desmin-related myopathy." Nat Genet 20(1): 92-95.
Viegas-Pequignot, E., Z. L. Li, B. Dutrillaux, F. Apiou and D. Paulin (1989). "Assignment of human desmin gene to band 2q35 by nonradioactive in situ hybridization." Hum Genet 83(1): 33-36.
Wang, X., R. Klevitsky, W. Huang, J. Glasford, F. Li and J. Robbins (2003). "AlphaB-crystallin modulates protein aggregation of abnormal desmin." Circ Res 93(10): 998-1005.
Weber, K. and N. Geisler (1985). "Intermediate filaments: structural conservation and divergence." Ann N Y Acad Sci 455: 126-143.
Weisleder, N., G. E. Taffet and Y. Capetanaki (2004). "Bcl-2 overexpression corrects mitochondrial defects and ameliorates inherited desmin null cardiomyopathy." Proc Natl Acad Sci U S A 101(3): 769-774.