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plectin; PCN; PLTN; hemidesmosomal protein 1; HD1; plectin-1 (PLEC PLEC1)
Function:
- interlinks intermediate filaments with microtubules & microfilaments
- anchors intermediate filaments to desmosomes or hemidesmosomes
- N-terminus interacts with actin, integrin beta-4
- C-terminus interacts with vimentin, desmin, GFAP, cytokeratins, lamin B, integrin beta-4
- phosphorylated by cdc2
- phosphorylated by CDK1; regulates dissociation from intermediate filaments during mitosis (putative)
- phosphorylated upon DNA damage, probably by ATM or ATR
- interacts (via actin-binding domain) with Nesprin-3
- interacts (via CH 1 domain) with VIM (via rod region)
Structure:
- homodimer or homotetramer
- the N-terminus interacts with actin
- the C-terminus interacts with vimentin, desmin, GFAP, cytokeratins, lamin B
- both the N-terminus & C-terminus can bind integrin beta-4
- belongs to the plakin or cytolinker family
- contains 1 actin-binding domain
- contains 2 CH (calponin-homology) domains
- contains 33 plectin repeats
- contains 4 spectrin repeats
Compartment:
- cytoplasm, cytoskeleton
Alternative splicing:
- named isoforms=9
- phosphorylated on Ser-21 & Tyr-26
Expression:
- widely expressed with highest levels in muscle, heart, placenta & spinal cord
Pathology:
- mutations associated with:
a) epidermolysis bullosa simplex with pyloric atresia
a) epidermolysis bullosa simplex with muscular dystrophy
b) epidermolysis bullosa simplex 1 (Ogna type)
General
cytoskeletal protein
phosphoprotein
Properties
SIZE: entity length = 4684 aa
MW = 532 kD
COMPARTMENT: cytoplasm
MOTIF: Globular {1-1470}
MOTIF: Ser phosphorylation site {S125}
Ser phosphorylation site {S149}
actin-binding site
SITE: 175-400
FOR-BINDING-OF: F-actin
calponin homology domain
NAME: calponin homology domain
SITE: 179-282
Ser phosphorylation site {S201}
calponin homology domain
NAME: calponin homology domain
SITE: 295-397
spectrin repeat {645-710}
Ser phosphorylation site {S720}
spectrin repeat {740-824}
Ser phosphorylation site {S782}
spectrin repeat {837-930}
spectrin repeat {1315-1415}
Ser phosphorylation site {S1435}
Ser phosphorylation site {S1444}
coiled coil {1469-2756}
MOTIF: Central fibrous rod {1471-2755}
Ser phosphorylation site {S1644}
Ser phosphorylation site {S1721}
Ser phosphorylation site {S1732}
Ser phosphorylation site {S2516}
Ser phosphorylation site {S2755}
Globular {2756-4684}
MOTIF: Thr phosphorylation site {T2814}
Plectin {2826-2863}
Plectin {2864-2901}
Plectin {2902-2939}
Plectin {2940-2977}
Plectin {2981-3015}
Tyr phosphorylation site {Y3033}
Plectin {3116-3153}
Plectin {3154-3191}
Plectin {3192-3229}
Plectin {3230-3267}
Plectin {3268-3305}
Plectin {3306-3343}
Tyr phosphorylation site {Y3362}
Plectin {3485-3522}
Plectin {3523-3560}
Plectin {3561-3598}
Plectin {3599-3636}
Plectin {3640-3674}
Plectin {3820-3857}
Plectin {3858-3895}
Plectin {3896-3933}
Plectin {3934-3971}
Plectin {3975-4008}
Thr phosphorylation site {T4030}
Plectin {4063-4100}
Plectin {4101-4138}
Plectin {4139-4176}
Tyr phosphorylation site {Y4155}
Plectin {4177-4214}
Plectin {4218-4252}
binding site
SITE: 4250-4300
FOR-BINDING-OF: intermediate filament
Plectin {4265-4305}
Plectin {4319-4356}
Ser phosphorylation site {S4382}
Ser phosphorylation site {S4384}
Ser phosphorylation site {S4385}
Ser phosphorylation site {S4386}
Ser phosphorylation site {S4389}
Ser phosphorylation site {S4390}
Ser phosphorylation site {S4391}
Ser phosphorylation site {S4392}
Tyr phosphorylation site {Y4393}
Ser phosphorylation site {S4396}
Ser phosphorylation site {S4400}
Thr phosphorylation site {T4402}
Plectin {4408-4445}
Thr phosphorylation site {T4411}
Plectin {4446-4483}
Plectin {4484-4521}
Plectin {4522-4559}
Thr phosphorylation site {T4539}
Plectin {4560-4597}
Tyr phosphorylation site {Y4611}
Ser phosphorylation site {S4613}
Tyr phosphorylation site {Y4615}
Ser phosphorylation site {S4618}
Ser phosphorylation site {S4620}
Ser phosphorylation site {S4622}
Thr phosphorylation site {T4623}
G-S-R-X {4625-4640}
Ser phosphorylation site {S4626}
Thr phosphorylation site {T4628}
Ser phosphorylation site {S4642}
Ser phosphorylation site {S4658}
Ser phosphorylation site {S4675}
Database Correlations
OMIM correlations
MORBIDMAP 601282
UniProt Q15149
PFAM correlations
Entrez Gene 5339
Kegg hsa:5339
References
- UniProt :accession Q15149
- Wikipedia; Note: plectin entry
http://en.wikipedia.org/wiki/plectin