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filamin B (beta filamin, actin binding protein-278, thyroid autoantigen, truncated actin-binding protein, truncated ABP, ABP-280 homolog, ABP-278, filamin 3, filamin homolog 1, Fh1, FLNB, FLN1L, FLN3, TABP TAP)
Function:
1) connects cell membrane constituents to the actin cytoskeleton
2) neuroblast migration from the ventricular zone into the cortical plate
3) myotube morphology & myogenesis
Compartment:
- cytoplasmic, peripheral (isoform 1)
- isoforms 2 & 3 are predominantly localized at actin stress fibers
Alternative splicing: named isoforms=6
Expression: ubiquitous
Pathology:
- interaction with FLNA may compensate for dysfunctional FLNA homodimer in the periventricular nodular heterotopia
General
filamin (actin-binding protein)
Properties
CONFIGURATION: dimer
SIZE: MW = 278 kD
entity length = 2602 aa
COMPARTMENT: cytoplasm
MOTIF: actin-binding site
SITE: 1-239
FOR-BINDING-OF: F-actin
MOTIF: calponin homology domain
NAME: calponin homology domain
SITE: 16-122
calponin homology domain
NAME: calponin homology domain
SITE: 139-239
Filamin Repeat {249-1222} (10)
binding site
SITE: 1128-1511
FOR-BINDING-OF: Filamin-binding LIM protein 1
MOTIF: Filamin Repeat {1223-1511} (3)
Filamin Repeat {1512-1704} (2)
Hinge 1 {1705-1728}
Filamin Repeat {1729-1908} (2)
binding site
SITE: 1862-2148
FOR-BINDING-OF: platelet glycoprotein Ib alpha chain
MOTIF: Filamin Repeat {1919-2089} (2)
binding site
SITE: 2060-2225
FOR-BINDING-OF: Filamin-A
MOTIF: Filamin Repeat {2091-2185}
interaction with INPPL1. {2130-2602}
MOTIF: consensus repeat {2188-2280} (3)
Hinge 2. {2472-2506}
Tyr phosphorylation site {Y2502}
Filamin Repeat {2507-2601}
binding site
SITE: 2507-2602
FOR-BINDING-OF: Filamin-A
Database Correlations
OMIM 603381
MORBIDMAP 603381
UniProt O75369
PFAM correlations
Entrez Gene 2317
References
- Entrez Gene :accession 2317
- UniProt :accession O75369