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LDB3 encodes a PDZ domain-containing protein. De plus, nous expédions LIM Domain Binding 3 Protein Anticorps (65) et LIM Domain Binding 3 Protein Protéines (5) et beaucoup plus de produits pour cette protéine.
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 (Montrer TCAP Kits ELISA) 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 (Montrer ACTN1 Kits ELISA) 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 (Montrer PDLIM5 Kits ELISA) domain for protein kinase C (Montrer PKC Kits ELISA), 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 (Montrer ACTN1 Kits ELISA) in cultured myoblasts and nonmuscle cells.
ZASP/Cypher anchors PGM1 (Montrer PGM1 Kits ELISA) to Z-disc under conditions of stress. The impaired binding of PGM1 (Montrer PGM1 Kits ELISA) to ZASP/Cypher might be involved in the pathogenesis of dilated cardiomyopathy.
Cypher and ENH (Montrer PDLIM5 Kits ELISA) 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 (Montrer TTN Kits ELISA) and vinculin (Montrer VCL Kits ELISA) preferentially bind to alpha-actinin-2 (Montrer ACTN2 Kits ELISA).
Deletion of long but not short Cypher isoforms leads to late-onset dilated cardiomyopathy.
mutations of a structural/cytoskeletal protein (Montrer ADD3 Kits ELISA), 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