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LDB3 encodes a PDZ domain-containing protein. 再加上，我们可以发LIM Domain Binding 3 Protein 蛋白 (5) 和 和数多这个蛋白质的别的产品。
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Human Polyclonal LIM Domain Binding 3 Protein Primary Antibody for IHC (p), IHC - ABIN250866
Huang, Zhou, Liang, Hollander, Sheikh, Li, Greaser, Shelton, Evans, Chen: Characterization and in vivo functional analysis of splice variants of cypher. in The Journal of biological chemistry 2003
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Human Monoclonal LIM Domain Binding 3 Protein Primary Antibody for IF, RNAi - ABIN524582
Arimura, Inagaki, Hayashi, Shichi, Sato, Hinohara, Vatta, Towbin, Chikamori, Yamashina, Kimura: Impaired binding of ZASP/Cypher with phosphoglucomutase 1 is associated with dilated cardiomyopathy. in Cardiovascular research 2009
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Dog (Canine) Polyclonal LIM Domain Binding 3 Protein Primary Antibody for ELISA, WB - ABIN547516
Selcen, Engel: Mutations in ZASP define a novel form of muscular dystrophy in humans. in Annals of neurology 2005
Interaction is demonstrated between ZASP and two new binding partners both of which have roles in signalling, regulation of gene expression and muscle differentiation; the mechanosensing protein Ankrd2 and the tumour suppressor protein p53.
p.(D117N) variant in Cypher/ZASP is not a causative mutation for dilated cardiomyopathy and ventricular arrhythmias.
A novel heterozygous missense mutation (p.N155H) in a highly conserved PDZ-like motif of ZASP was identified. The results indicate that typical ZASP-MFM presenting with late-onset distal myopathy is commonly associated with mutations in PDZ-like motif of ZASP.
The molecular basis for high-affinity binding of ZASP to G-Actin has been described.
this is the first family with Arrhythmogenic right ventricular cardiomyopathy where a mutation in LDB3 is associated with Arrhythmogenic right ventricular cardiomyopathy
Study identified abnormal inclusion of LDB3 exon 11 specific to myotonic dystrophy type at the RNA and protein level; inclusion changed the affinity of LDB3 for PKC, indicating that exon 11 may contribute to the activation of PKC in DM1
results show that MFM-associated ZASP mutations in the actin-binding domain have deleterious effects on the core structure of the Z-discs in skeletal muscle.
Z-band alternatively spliced PDZ motif protein (ZASP) is the major O-linked beta-N-acetylglucosamine-substituted protein in human heart myofibrils
This results of this indicted that One patient harbored the missense mutation c.1719G>A (p.V566M) in the ZASP gene.
ZASP1 can form protein complex with telethonin/T-Cap and Na(v)1.5. The left ventricular noncompaction-specific ZASP1 mutation can cause loss of function of Na(v)1.5, without significant alteration of the cytoskeletal protein complex.
TNNT3 and LDB3 showed abnormal splicing and appeared more pronounced in myotonic dystrophies type 2 relative to myotonic dystrophies type 1.
the D626N mutation of Cypher/ZASP increases the affinity of the LIM domain for protein kinase C, which may be related to pathogenesis of dilated cardiomyopathy
ZASP/Cypher internal fragments containing either ZM exon 4 or 6 co-localized with alpha-actinin in cultured myoblasts and nonmuscle cells.
Tafazzins are essential during fetal and early post-natal life.
ZASP/Cypher anchors PGM1 to Z-disc under conditions of stress. The impaired binding of PGM1 to ZASP/Cypher might be involved in the pathogenesis of dilated cardiomyopathy.
Cypher and ENH redundantly play an essential role in sustaining Z-line structure from the earliest stages of cardiac function, and are redundantly required to maintain normal embryonic heart function and embryonic viability.
Results indicte that Cypher/ZASP interacted with the regulatory subunit RIIalpha of PKA.
Data demonstrated that the Z-disk proteins, ZASP, titin and vinculin preferentially bind to alpha-actinin-2.
Deletion of long but not short Cypher isoforms leads to late-onset dilated cardiomyopathy.
mutations of a structural/cytoskeletal protein, such as ZASP, lead to cardiac functional and electric abnormalities.
Cypher isoforms are developmentally regulated in both skeletal and cardiac muscle
Cypher plays a pivotal role in adult cardiac structure and function through protein-protein interactions with other Z-line proteins.
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.
LIM domain binding 3
, LIM domain binding 3 like
, LIM domain-binding protein 3
, PDZ and LIM domain 6
, Z-band alternatively spliced PDZ-motif protein
, protein cypher
, PDZ-LIM domain protein
, oracle protein
, protein oracle