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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/11/26 14:56:07」(JST)

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Plectin is a giant protein found in nearly all mammalian cells which acts as a link between the three main components of the cytoskeleton: actin microfilaments, microtubules and intermediate filaments.^[1] In addition plectin links the cytoskeleton to junctions found in the plasma membrane that structurally connect different cells. By holding these different networks together plectin plays an important role in maintaining the mechanical integrity and viscoelastic properties of tissues.^[2]

Structure

Plectin can exist in cells as several alternatively-spliced isoforms, all around 500 kDa and >4000 amino acids.^[3]^[4] The structure of plectin is thought to be a dimer consisting of a central coiled coil of alpha helices connecting two large globular domains (one at each terminus). These globular domains are responsible for connecting plectin to its various cytoskeletal targets. The carboxy-terminal domain is made of 6 highly homologous repeating regions. The subdomain between regions five and six of this domain is known to connect to the intermediate filaments cytokeratin and vimentin. At the opposite end of the protein, in the N-terminal domain, a region has been defined as responsible for binding to actin.^[5] In 2004, the exact crystal structure of this actin-binding domain (ABD) was determined in mice and shown to be composed of two calponin homology (CH) domains.^[6] Plectin is expressed in nearly all mammalian tissues. In cardiac muscle and skeletal muscle, plectin is localized to specialized entities known as Z-discs.^[7] Plectin binds several proteins, including vinculin, DES,^[8] actin.,^[2]^[9] fodrin,^[2]^[9] microtubule-associating proteins,^[2]^[9] nuclear laminin B.,^[2]^[9] SPTAN1,^[10]^[11] vimentin^[10]^[11]^[12] and ITGB4.^[2]^[9]

Function

Studies employing a plectin knockout mouse have shed light on the functions of plectin. Pups died 2–3 days after birth, and these mice exhibited marked skin abnormalities, including degeneration of keratinocytes. Skeletal and cardiac muscle tissues were also significantly affected. Cardiac intercalated discs were disintegrated and sarcomeres were irregularly shapen, and intracellular accumulation of aberrant isolated myofibrillar bundles and Z-disc components was also observed. Expression of vinculin in muscle cells was strikingly down-regulated.^[13] Through the use of gold-immunoelectron microscopy, immunoblotting and immunofluorescence experiments plectin has been found to associate with all three major components of the cytoskeleton. In muscle, plectin binds to the periphery of Z-discs,^[8] and along with the intermediate filament protein desmin, may form lateral linkages among neighboring Z-discs. This interaction between plectin and desmin intermediate filaments also appears to faclitate the close association of myofibrils and mitochondria, both at Z-discs and along the remainder the sarcomere.^[14] Plectin also functions to link cytoskeleton to intercellular junctions, such as desmosomes and hemidesmosomes, which link intermediate filament networks between cells. Plectin has been revealed to localize to the desmosomes and in vitro studies have shown that it can form bridges between the desmosome protein, desmoplakin and intermediate filaments.^[15] In hemidesmosomes plectin has been shown to interact with the integrin β4 subunits of the hemidesmosome plaque and function in a clamp like manner to crosslink the intermediate filament, cytokeratin to the junction.^[16]

Clinical Significance

Mutations in PLEC have been associated with epidermolysis bullosa simplex with muscular dystrophy. Isolated left ventricular non-compaction accompanying epidermolysis bullosa simplex with muscular dystrophy was also noted.^[17] Plectin has been proposed as a biomarker for pancreatic cancer.^[18]^[19] Although normally a cytoplasmic protein, plectin is expressed on the cell membrane in pancreatic ductal adenocarcinoma (PDAC) and can therefore be used to target PDAC cells.^[18]

References

^ Svitkina TM, Verkhovsky AB, Borisy GG (Nov 1996). "Plectin sidearms mediate interaction of intermediate filaments with microtubules and other components of the cytoskeleton". The Journal of Cell Biology 135 (4): 991–1007. doi:10.1083/jcb.135.4.991. PMC 2133373. PMID 8922382.
^ ^a ^b ^c ^d ^e ^f Wiche G (Sep 1998). "Role of plectin in cytoskeleton organization and dynamics" (abstract). Journal of Cell Science. 111 ( Pt 17) (17): 2477–86. PMID 9701547.
^ http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=Q15149-2. Missing or empty |title= (help)
^ Zong, N. C.; Li, H; Li, H; Lam, M. P.; Jimenez, R. C.; Kim, C. S.; Deng, N; Kim, A. K.; Choi, J. H.; Zelaya, I; Liem, D; Meyer, D; Odeberg, J; Fang, C; Lu, H. J.; Xu, T; Weiss, J; Duan, H; Uhlen, M; Yates Jr, 3rd; Apweiler, R; Ge, J; Hermjakob, H; Ping, P (2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research 113 (9): 1043–53. doi:10.1161/CIRCRESAHA.113.301151. PMC 4076475. PMID 23965338.
^ Winter L, Wiche G (Jan 2013). "The many faces of plectin and plectinopathies: pathology and mechanisms". Acta Neuropathologica 125 (1). doi:10.1007/s00401-012-1026-0. PMID 22864774.
^ Sevcík J, Urbániková L, Kost'an J, Janda L, Wiche G (May 2004). "Actin-binding domain of mouse plectin. Crystal structure and binding to vimentin". European Journal of Biochemistry / FEBS 271 (10): 1873–84. doi:10.1111/j.1432-1033.2004.04095.x. PMID 15128297.
^ Zernig G, Wiche G (Jul 1985). "Morphological integrity of single adult cardiac myocytes isolated by collagenase treatment: immunolocalization of tubulin, microtubule-associated proteins 1 and 2, plectin, vimentin, and vinculin". European Journal of Cell Biology 38 (1). PMID 2992982.
^ ^a ^b Hijikata T, Murakami T, Imamura M, Fujimaki N, Ishikawa H (Mar 1999). "Plectin is a linker of intermediate filaments to Z-discs in skeletal muscle fibers". Journal of Cell Science. 112 ( Pt 6). PMID 10036236.
^ ^a ^b ^c ^d ^e Steinböck FA, Wiche G (Feb 1999). "Plectin: a cytolinker by design". Biological Chemistry 380 (2). doi:10.1515/BC.1999.023. PMID 10195422.
^ ^a ^b Herrmann H, Wiche G (Jan 1987). "Plectin and IFAP-300K are homologous proteins binding to microtubule-associated proteins 1 and 2 and to the 240-kilodalton subunit of spectrin". The Journal of Biological Chemistry 262 (3): 1320–5. PMID 3027087.
^ ^a ^b Brown MJ, Hallam JA, Liu Y, Yamada KM, Shaw S (Jul 2001). "Cutting edge: integration of human T lymphocyte cytoskeleton by the cytolinker plectin". Journal of Immunology 167 (2): 641–5. doi:10.4049/jimmunol.167.2.641. PMID 11441066.
^ Favre B, Schneider Y, Lingasamy P, Bouameur JE, Begré N, Gontier Y, Steiner-Champliaud MF, Frias MA, Borradori L, Fontao L (May 2011). "Plectin interacts with the rod domain of type III intermediate filament proteins desmin and vimentin". European Journal of Cell Biology 90 (5). doi:10.1016/j.ejcb.2010.11.013. PMID 21296452.
^ Andrä K, Lassmann H, Bittner R, Shorny S, Fässler R, Propst F, Wiche G (Dec 1997). "Targeted inactivation of plectin reveals essential function in maintaining the integrity of skin, muscle, and heart cytoarchitecture". Genes & Development 11 (23). PMID 9389647.
^ Reipert S, Steinböck F, Fischer I, Bittner RE, Zeöld A, Wiche G (Nov 1999). "Association of mitochondria with plectin and desmin intermediate filaments in striated muscle". Experimental Cell Research 252 (2). doi:10.1006/excr.1999.4626. PMID 10527638.
^ Huber O (Sep 2003). "Structure and function of desmosomal proteins and their role in development and disease". Cellular and Molecular Life Sciences 60 (9). doi:10.1007/s00018-003-3050-7. PMID 14523549.
^ Sonnenberg A, Liem RK (Jun 2007). "Plakins in development and disease". Experimental Cell Research 313 (10). doi:10.1016/j.yexcr.2007.03.039. PMID 17499243.
^ Villa CR, Ryan TD, Collins JJ, Taylor MD, Lucky AW, Jefferies JL (Feb 2015). "Left ventricular non-compaction cardiomyopathy associated with epidermolysis bullosa simplex with muscular dystrophy and PLEC1 mutation". Neuromuscular Disorders 25 (2). doi:10.1016/j.nmd.2014.09.011. PMID 25454730.
^ ^a ^b Kelly KA, Bardeesy N, Anbazhagan R, Gurumurthy S, Berger J, Alencar H, Depinho RA, Mahmood U, Weissleder R (Apr 2008). "Targeted nanoparticles for imaging incipient pancreatic ductal adenocarcinoma". PLoS Medicine 5 (4): e85. doi:10.1371/journal.pmed.0050085. PMC 2292750. PMID 18416599.
^ Bausch D, Thomas S, Mino-Kenudson M, Fernández-del CC, Bauer TW, Williams M, Warshaw AL, Thayer SP, Kelly KA (Jan 2011). "Plectin-1 as a novel biomarker for pancreatic cancer". Clinical Cancer Research 17 (2): 302–9. doi:10.1158/1078-0432.CCR-10-0999. PMC 3044444. PMID 21098698.

External links

Mass spectrometry characterization of one isoform of PLEC at COPaKB.^[1]
GeneReviews/NCBI/NIH/UW entry on Epidermolysis Bullosa with Pyloric Atresia
plectin at the US National Library of Medicine Medical Subject Headings (MeSH)
"Plectin". The University of Edinburgh. 2003-11-13. Retrieved 2008-02-17.

^ Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P (Oct 2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research 113 (9). doi:10.1161/CIRCRESAHA.113.301151. PMID 23965338.

UpToDate Contents

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1. 表皮水疱症の疫学、病因、分類、および臨床的特徴 epidemiology pathogenesis classification and clinical features of epidermolysis bullosa
2. 腫瘍随伴性天疱瘡 paraneoplastic pemphigus
3. 水疱性類天疱瘡および粘膜類天疱瘡の疫学および病因 epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid

English Journal

Vimentin as a Marker of Early Differentiating, Highly Motile Corneal Epithelial Cells.

Castro-Muñozledo F1, Meza-Aguilar DG1, Domínguez-Castillo R2, Hernández-Zequinely V1, Sánchez-Guzmán E1.
Journal of cellular physiology.J Cell Physiol.2017 Apr;232(4):818-830. doi: 10.1002/jcp.25487. Epub 2016 Jul 20.
Vimentin (Vim), a cytoskeletal intermediate filament, is part of a naturally occurring reversible program, the Epithelial-Mesenchymal Transition (EMT), which converts epithelial cells into mesenchymal-like derivatives. Based on previous results showing that epithelial cells co-express Vim and kerati
PMID 27404216

A Cyclin D2-derived peptide acts on specific cell cycle phases by activating ERK1/2 to cause the death of breast cancer cells.

Russo LC1, Araujo CB2, Iwai LK3, Ferro ES4, Forti FL5.
Journal of proteomics.J Proteomics.2017 Jan 16;151:24-32. doi: 10.1016/j.jprot.2016.06.028. Epub 2016 Jun 29.
Protein degradation by the proteasome generates functional intracellular peptides. Pep5, a peptide derived from Cyclin D2, induces cell death in tumor cell lines and reduces the volume of rat C6 glioblastoma tumors in vivo. Here, we chose the human MDA-MB-231 breast cancer cells to evaluate the mech
PMID 27371349

Epidermolysis Bullosa with Pyloric Atresia and Significant Urologic Involvement.

Walker GD1, Woody M2, Orrin E3, Mellerio JE3, Levy ML2,4.
Pediatric dermatology.Pediatr Dermatol.2017 Jan;34(1):e61-e64. doi: 10.1111/pde.13026. Epub 2016 Nov 4.
Epidermolysis bullosa (EB) is a rare inherited disease that causes epidermal fragility, blistering, and erosions. EB results from a variety of mutations in proteins of the skin and mucous membranes of the body. Mutations in plectin a protein involved in hemidesmosome integrity and function, are asso
PMID 27813154

Japanese Journal

Plectin is a novel regulator for apical extrusion of RasV12-transformed cells

Scientific Reports 7, 44328, 2017-03-10
NAID 120006219881

3D04 血管平滑筋細胞内ストレスファイバの配向再現メカニズムに関する研究 : 中間径フィラメントおよび細胞骨格リンカータンパク質との相互作用(GS1-3:細胞のバイオメカニクス(3))

バイオエンジニアリング講演会講演論文集 2013(25), 559-560, 2013-01-08
NAID 110009920159

Plectin deficiency on cytoskeletal disorganization and transformation of human liver cells in vitro

Medical molecular morphology : official journal of the Japanese Society for Clinical Molecular Morphology 44(1), 21-26, 2011-03-01
NAID 10029036494

Related Pictures

Plectin Plectin reinforces vascular integrity by mediating crosstalk between the vimentin and Plectin Antibody (10F6) | SCBT - Santa Cruz Biotechnology Lecture 7: the cytoskeleton and cell movement (Microtubules and intermediate filaments Phosphoproteins Plectin-1 (D6A11) Rabbit mAb #12254 | CST 抗体 Plectin 1 links intermediate filaments to costameric sarcolemma through β-synemin, α Analysis of the interactions between BP180, BP230, plectin and the integrin α6β4

★リンクテーブル★

リンク元

「プレクチン」

Plectin
	PDB rendering based on 1mb8.
Available structures
PDB	Ortholog search: PDBe, RCSB
List of PDB id codes
	1MB8, 2ODU, 2ODV, 3F7P, 3PDY, 3PE0, 4GDO, 4Q58, 4Q59
Identifiers
Symbols	PLEC ; EBS1; EBSO; HD1; LGMD2Q; PCN; PLEC1; PLEC1b; PLTN
External IDs	OMIM: 601282 MGI: 1277961 HomoloGene: 384 ChEMBL: 1293240 GeneCards: PLEC Gene
Gene ontology
Molecular function	• actin binding; • protein binding; • structural constituent of muscle; • ankyrin binding; • poly(A) RNA binding;
Cellular component	• cytoplasm; • cytosol; • plasma membrane; • brush border; • focal adhesion; • sarcoplasm; • hemidesmosome; • sarcolemma; • costamere; • intermediate filament cytoskeleton; • extracellular exosome;
Biological process	• apoptotic process; • cellular component disassembly involved in execution phase of apoptosis; • programmed cell death; • extracellular matrix organization; • hemidesmosome assembly; • cell junction assembly;
	Sources: Amigo / QuickGO
Orthologs
	v; t; e;

　　[★]

英: plectin
関: [[]]

ビメンチンと結合し、細胞内で三次元的網目構造を作る (HIS.40)
プレクチンは表皮基底膜ヘミデスモソームと筋膜サルコレンマに存在 (NDE.205)

臨床関連

プレクチン欠損症

[pmid8922382-1] Svitkina TM, Verkhovsky AB, Borisy GG (Nov 1996). "Plectin sidearms mediate interaction of intermediate filaments with microtubules and other components of the cytoskeleton". The Journal of Cell Biology 135 (4): 991–1007. doi:10.1083/jcb.135.4.991. PMC 2133373. PMID 8922382.

[pmid9701547-2] ^ ^a ^b ^c ^d ^e ^f Wiche G (Sep 1998). "Role of plectin in cytoskeleton organization and dynamics" (abstract). Journal of Cell Science. 111 ( Pt 17) (17): 2477–86. PMID 9701547.

[3] ttp://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=Q15149-2. Missing or empty |title= (help)

[4] Zong, N. C.; Li, H; Li, H; Lam, M. P.; Jimenez, R. C.; Kim, C. S.; Deng, N; Kim, A. K.; Choi, J. H.; Zelaya, I; Liem, D; Meyer, D; Odeberg, J; Fang, C; Lu, H. J.; Xu, T; Weiss, J; Duan, H; Uhlen, M; Yates Jr, 3rd; Apweiler, R; Ge, J; Hermjakob, H; Ping, P (2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research 113 (9): 1043–53. doi:10.1161/CIRCRESAHA.113.301151. PMC 4076475. PMID 23965338.

[pmid22864774-5] Winter L, Wiche G (Jan 2013). "The many faces of plectin and plectinopathies: pathology and mechanisms". Acta Neuropathologica 125 (1). doi:10.1007/s00401-012-1026-0. PMID 22864774.

[pmid15128297-6] Sevcík J, Urbániková L, Kost'an J, Janda L, Wiche G (May 2004). "Actin-binding domain of mouse plectin. Crystal structure and binding to vimentin". European Journal of Biochemistry / FEBS 271 (10): 1873–84. doi:10.1111/j.1432-1033.2004.04095.x. PMID 15128297.

[pmid2992982-7] Zernig G, Wiche G (Jul 1985). "Morphological integrity of single adult cardiac myocytes isolated by collagenase treatment: immunolocalization of tubulin, microtubule-associated proteins 1 and 2, plectin, vimentin, and vinculin". European Journal of Cell Biology 38 (1). PMID 2992982.

[pmid10036236-8] Hijikata T, Murakami T, Imamura M, Fujimaki N, Ishikawa H (Mar 1999). "Plectin is a linker of intermediate filaments to Z-discs in skeletal muscle fibers". Journal of Cell Science. 112 ( Pt 6). PMID 10036236.

[pmid10195422-9] Steinböck FA, Wiche G (Feb 1999). "Plectin: a cytolinker by design". Biological Chemistry 380 (2). doi:10.1515/BC.1999.023. PMID 10195422.

[pmid3027087-10] Herrmann H, Wiche G (Jan 1987). "Plectin and IFAP-300K are homologous proteins binding to microtubule-associated proteins 1 and 2 and to the 240-kilodalton subunit of spectrin". The Journal of Biological Chemistry 262 (3): 1320–5. PMID 3027087.

[pmid11441066-11] Brown MJ, Hallam JA, Liu Y, Yamada KM, Shaw S (Jul 2001). "Cutting edge: integration of human T lymphocyte cytoskeleton by the cytolinker plectin". Journal of Immunology 167 (2): 641–5. doi:10.4049/jimmunol.167.2.641. PMID 11441066.

[pmid21296452-12] Favre B, Schneider Y, Lingasamy P, Bouameur JE, Begré N, Gontier Y, Steiner-Champliaud MF, Frias MA, Borradori L, Fontao L (May 2011). "Plectin interacts with the rod domain of type III intermediate filament proteins desmin and vimentin". European Journal of Cell Biology 90 (5). doi:10.1016/j.ejcb.2010.11.013. PMID 21296452.

[pmid9389647-13] Andrä K, Lassmann H, Bittner R, Shorny S, Fässler R, Propst F, Wiche G (Dec 1997). "Targeted inactivation of plectin reveals essential function in maintaining the integrity of skin, muscle, and heart cytoarchitecture". Genes & Development 11 (23). PMID 9389647.

[pmid10527638-14] Reipert S, Steinböck F, Fischer I, Bittner RE, Zeöld A, Wiche G (Nov 1999). "Association of mitochondria with plectin and desmin intermediate filaments in striated muscle". Experimental Cell Research 252 (2). doi:10.1006/excr.1999.4626. PMID 10527638.

[pmid14523549-15] Huber O (Sep 2003). "Structure and function of desmosomal proteins and their role in development and disease". Cellular and Molecular Life Sciences 60 (9). doi:10.1007/s00018-003-3050-7. PMID 14523549.

[pmid17499243-16] Sonnenberg A, Liem RK (Jun 2007). "Plakins in development and disease". Experimental Cell Research 313 (10). doi:10.1016/j.yexcr.2007.03.039. PMID 17499243.

[pmid25454730-17] Villa CR, Ryan TD, Collins JJ, Taylor MD, Lucky AW, Jefferies JL (Feb 2015). "Left ventricular non-compaction cardiomyopathy associated with epidermolysis bullosa simplex with muscular dystrophy and PLEC1 mutation". Neuromuscular Disorders 25 (2). doi:10.1016/j.nmd.2014.09.011. PMID 25454730.

[pmid18416599-18] Kelly KA, Bardeesy N, Anbazhagan R, Gurumurthy S, Berger J, Alencar H, Depinho RA, Mahmood U, Weissleder R (Apr 2008). "Targeted nanoparticles for imaging incipient pancreatic ductal adenocarcinoma". PLoS Medicine 5 (4): e85. doi:10.1371/journal.pmed.0050085. PMC 2292750. PMID 18416599.

[pmid21098698-19] Bausch D, Thomas S, Mino-Kenudson M, Fernández-del CC, Bauer TW, Williams M, Warshaw AL, Thayer SP, Kelly KA (Jan 2011). "Plectin-1 as a novel biomarker for pancreatic cancer". Clinical Cancer Research 17 (2): 302–9. doi:10.1158/1078-0432.CCR-10-0999. PMC 3044444. PMID 21098698.

[pmid23965338-20] Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P (Oct 2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research 113 (9). doi:10.1161/CIRCRESAHA.113.301151. PMID 23965338.

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plectin