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LIM domain-binding protein 3 (Z-band alternatively spliced PDZ-motif protein, protein cypher, LDB3, KIAA0613, ZASP)
Function:
1) adapter in striated muscle to couple protein kinase C- mediated signaling via its LIM domains to the cytoskeleton
2) interacts with ACTN2 C-terminal region,MYOZ1, MYOZ2, MYOZ3
Compartment:
- localized to the cytoplasm around nuclei, pseudopodia of undifferentiated cells & myotubes of differentiated cells
- colocalizes with ACTN2 at the Z-lines
Alternative splicing: named isoforms=6
Expression:
- expressed primarily in skeletal muscle > heart, brain, placenta
Pathology:
- defects in LDB3 are a cause of dilated cardiomyopathy 1A with or without left ventricular non-compaction
- defects in LDB3 are the cause of ZASP-related myofibrillar myopathy distal more than proximal muscle weakness with signs of cardiomyopathy and neuropathy.
General
LIM domain-binding protein
Properties
SIZE: MW = 77 kD
entity length = 727 aa
COMPARTMENT: cell nucleus
MOTIF: PDZ domain
NAME: PDZ domain
SITE: 1-84
binding site
SITE: 549-607
FOR-BINDING-OF: transcription factor
FOR-BINDING-VIA: LIM domain
binding site
SITE: 608-667
FOR-BINDING-OF: transcription factor
FOR-BINDING-VIA: LIM domain
binding site
SITE: 668-727
FOR-BINDING-OF: transcription factor
FOR-BINDING-VIA: LIM domain
Database Correlations
OMIM correlations
MORBIDMAP 605906
UniProt O75112
PFAM correlations
References
UniProt :accession O43679