NP_001073583.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
78,504 Da
NCBI Official Full Name
LIM domain-binding protein 3 isoform 2
NCBI Official Synonym Full Names
LIM domain binding 3
NCBI Official Synonym Symbols
MFM4; ZASP; CMD1C; CMPD3; LVNC3; CYPHER; LDB3Z1; LDB3Z4; ORACLE; PDLIM6 [Similar Products]
NCBI Protein Information
LIM domain-binding protein 3; LDB3; protein cypher; PDZ and LIM domain 6; Z-band alternatively spliced PDZ motif protein; Z-band alternatively spliced PDZ-motif protein; cardiomyopathy, dilated 1C (autosomal dominant)
UniProt Protein Name
LIM domain-binding protein 3
UniProt Synonym Protein Names
Protein cypher; Z-band alternatively spliced PDZ-motif protein
UniProt Entry Name
LDB3_HUMAN
NCBI Summary for LDB3
This gene encodes a PDZ domain-containing protein. PDZ motifs are modular protein-protein interaction domains consisting of 80-120 amino acid residues. PDZ domain-containing proteins interact with each other in cytoskeletal assembly or with other proteins involved in targeting and clustering of membrane proteins. The protein encoded by this gene interacts with alpha-actinin-2 through its N-terminal PDZ domain and with protein kinase C via its C-terminal LIM domains. The LIM domain is a cysteine-rich motif defined by 50-60 amino acids containing two zinc-binding modules. This protein also interacts with all three members of the myozenin family. Mutations in this gene have been associated with myofibrillar myopathy and dilated cardiomyopathy. Alternatively spliced transcript variants encoding different isoforms have been identified; all isoforms have N-terminal PDZ domains while only longer isoforms (1, 2 and 5) have C-terminal LIM domains. [provided by RefSeq, Jan 2010]
UniProt Comments for LDB3
LDB3: May function as an adapter in striated muscle to couple protein kinase C-mediated signaling via its LIM domains to the cytoskeleton. Defects in LDB3 are the cause of cardiomyopathy dilated type 1C (CMD1C). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. Defects in LDB3 are the cause of left ventricular non- compaction type 3 (LVNC3). Left ventricular non- compaction is characterized by numerous prominent trabeculations and deep intertrabecular recesses in hypertrophied and hypokinetic segments of the left ventricle. Defects in LDB3 are the cause of myopathy myofibrillar type 4 (MFM4). A neuromuscular disorder characterized by distal and proximal muscle weakness with signs of cardiomyopathy and neuropathy. 7 isoforms of the human protein are produced by alternative splicing.
Protein type: Cytoskeletal
Chromosomal Location of Human Ortholog: 10q22.3-q23.2
Cellular Component: cytoskeleton; perinuclear region of cytoplasm; pseudopodium; Z disc
Molecular Function: protein binding; protein kinase C binding; zinc ion binding; cytoskeletal protein binding; muscle alpha-actinin binding
Biological Process: sarcomere organization
Disease: Myopathy, Myofibrillar, 4
Research Articles on LDB3
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Diseases associated with anti-LDB3 antibody
Disease Name |
Pubmed Publications |
Hypertrophy Antibodies |
>7 publications with LDB3 and Hypertrophy |
Abnormalities, Multiple Antibodies |
>4 publications with LDB3 and Abnormalities, Multiple |
Atrophy Antibodies |
>4 publications with LDB3 and Atrophy |
Fibrosis Antibodies |
>3 publications with LDB3 and Fibrosis |
Necrosis Antibodies |
>3 publications with LDB3 and Necrosis |
MYOPATHY, MYOFIBRILLAR, 4 Antibodies |
>3 publications with LDB3 and MYOPATHY, MYOFIBRILLAR, 4 |
Brain Diseases Antibodies |
>2 publications with LDB3 and Brain Diseases |
Inflammation Antibodies |
>1 publications with LDB3 and Inflammation |
Kidney Diseases Antibodies |
>1 publications with LDB3 and Kidney Diseases |
Cardiomyopathy, Dilated, 1C Antibodies |
>1 publications with LDB3 and Cardiomyopathy, Dilated, 1C |
Organs/Tissues associated with anti-LDB3 antibody
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