BD-PN2473

LDB3 Rabbit Polyclonal Antibody

常规抗体

LDB3 KIAA0613 ZASP

LIM domain-binding protein 3 (Protein cypher) (Z-band alternatively spliced PDZ-motif protein)

WB

Human,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.

11155

O75112

https://www.uniprot.org/uniprotkb/O75112/entry

Q9JKS4

http://www.uniprot.org/uniprot/Q9JKS4

Synthesized peptide derived from human protein . at AA range: 41-90

LDB3 Polyclonal Antibody detects endogenous levels of protein.

WB 1:500-2000 ELISA 1:5000-20000

79kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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 (

Expressed primarily in skeletal muscle and to a lesser extent in heart. Also detected in brain and placenta.

Cytoplasm, perinuclear region . Cell projection, pseudopodium . Cytoplasm, cytoskeleton . Cytoplasm, myofibril, sarcomere, Z line . Localized to the cytoplasm around nuclei and pseudopodia of undifferentiated cells and detected throughout the myotubes of differentiated cells. Colocalizes with ACTN2 at the Z-lines.

disease:Defects in LDB3 are a cause of dilated cardiomyopathy with left ventricular non-compaction [MIM:601493]. Left ventricular non-compaction is characterized by numerous prominent trabeculations and deep intertrabecular recesses in hypertrophied and hypokinetic segments of the left ventricle.,disease:Defects in LDB3 are the cause of cardiomyopathy dilated type 1C (CMD1C) [MIM:601493]. 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.,disease:Defects in LDB3 are the cause of ZASP-related myofibrillar myopathy (MFM) [MIM:609452]. It is an autosomal dominant MFM that is characterized by distal more than proximal muscle weakness with signs of cardiomyopathy and neuropathy.,function:May function as an adapter in striated muscle to couple protein kinase C-mediated signaling via its LIM domains to the cytoskeleton.,similarity:Contains 1 PDZ (DHR) domain.,similarity:Contains 3 LIM zinc-binding domains.,subcellular location:Localized to the cytoplasm around nuclei and pseudopodia of undifferentiated cells and detected throughout the myotubes of differentiated cells. Colocalizes with ACTN2 at the Z-lines.,subunit:Interacts via its LIM domains with various PKC isoforms (By similarity). Interacts via its PDZ domain with the ACTN2 C-terminal region. Interacts with MYOZ1, MYOZ2 and MYOZ3.,tissue specificity:Expressed primarily in skeletal muscle and to a lesser extent in heart. Also detected in brain and placenta.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.