Dominant negative effect
Overcoming the Dominant Negative Effect of Mutant p53+/R172H Mice.
... a dominant negative effect over the remaining wild type allele in cells with one mutant and one wild type p53 allele (p53 +/Mut ) [176, 181, ...
93
Dominant negative effect of a mutant cardiac troponin T on cardiac structure and function in transgenic mice
... While mutations in the sarcomeric proteins have been shown to be the etiology of HCM, the mechanism responsible for HCM phenotypes remains unknown. Experimental stud- ies, in conjunction with clinical observations, ...
9
New insights on the mechanism(s) of the dominant negative effect of mutant thyroid hormone receptor in generalized resistance to thyroid hormone
... Generalized resistance to thyroid hormone (GRTH) is a syndrome of hyposensitivity to triiodothyronine (T3) that displays autosomal dominant inheritance. The genetic defect commonly lies in the ligand-binding ...
8
Dominant negative effect of the heterozygous C104R TACI mutation in common variable immunodeficiency (CVID)
... autosomal dominant auto- immune lymphoproliferative syndrome ...the dominant effect of the C76R mutant is that inclusion of the mutant in the preassociated TACI oligomeric complex would interfere ...
9
The dominant-negative interplay between p53, p63 and p73: A family affair
... Although scarcely found mutated in cancers, p63 and p73 genes encode multiple isoforms, some of which have been reported to exert a dominant-negative effect [20, 25, 51]. We first used FASAY strains ...
16
Genetic Interaction Between Integrins and moleskin, a Gene Encoding a Drosophila Homolog of Importin-7
... a dominant negative effect, but is due to inappropriate expression of functional integrins: wing blisters are not generated efficiently by misexpression of loss-of-function ␣PS2 subunits with ...
12
Dominant negative mutant Cyclin T1 proteins inhibit HIV transcription by specifically degrading Tat
... their dominant negative effect on HIV transcrip- tion by co-transfecting HeLa cells stably expressing the HIV-Luc reporter gene (HeLa/HR-Luc cells) with Tat (Fig- ...
12
Domain Structure of Lassa Virus L Protein
... various negative-strand RNA viruses, including paramyxoviruses (32, 33), LCMV (28), and Rift valley fever virus (40), as well as the heterotrimeric polymerase complex of influenza virus (19) form oligomers, and ...
10
A missense mutation in the Kv1 1 voltage gated potassium channel–encoding gene KCNA1 is linked to human autosomal dominant hypomagnesemia
... ous pore, forming a so-called tetrameric structure (25, 26). It has been shown that Kv1.1 channel subunits can assemble with other Kv channel subunits to form heterotetramers (27). Importantly, hypomagnesemia in the ...
8
Mutations in the glycosyltransferase domain of GLT8D1 are associated with familial amyotrophic lateral sclerosis
... common effect on this ...a dominant-negative effect with a competitive antagonism of wild-type enzyme ...example dominant-negative mutations in the GTPase Ras increase its ...
16
Interaction of Kaposi's Sarcoma-Associated Herpesvirus ORF59 with oriLyt Is Dependent on Binding with K-Rta
... a dominant negative effect on oriLyt amplification, suggesting that the interaction of K-Rta with ORF59 is essential for DNA synthesis and supporting the hypothesis that K-Rta facilitates the ...
9
Separating planar cell polarity and Hippo pathway activities of the protocadherins Fat and Dachsous
... any effect on ban3-GFP in wild-type discs, although we could weakly disrupt crossvein spacing and wing shape by hh- or en-gal4-driven expression of DsECD (Matakatsu and Blair, ...weaker effect in cells ...
11
Dominant-negative inhibitors of EBNA-1 of Epstein-Barr virus.
... the dominant-negative mu- tants of EBNA-1 are likely to inhibit wild-type EBNA-1 when they occupy only a subset of the binding sites on ...its dominant-negative mutants were intro- duced ...
10
The DNA resection protein CtIP promotes mammary tumorigenesis
... To confirm that CtIP lacks tumor suppression activity, we also examined the effect of Ctip ablation on mammary tumors induced by p53 deficiency. Surprisingly, in this setting Ctip inactivation dramatically reduced ...
12
Adenovirus-directed Expression of Dominant Negative Estrogen Receptor Induces Apoptosis in Breast Cancer Cells and Regression of Tumors in Nude Mice
... that dominant nega- tive ER mutants have the potential to induce apoptosis of T47D cells and regression of ...inant negative ERs by adenoviral vectors may provide a useful tool for targeted therapy of ...
10
Targeted inhibition of p38 MAPK promotes hypertrophic cardiomyopathy through upregulation of calcineurin NFAT signaling
... defect in NFAT activation in both the heart and T cells (16, 17). Thus, calcineurin gene targeting should potentially counteract the enhanced NFAT-activation profile and hypertrophic phenotype associated with p38 ...
13
Slit-Roundabout Signaling Neutralizes Netrin-Frazzled-Mediated Attractant Cue to Specify the Lateral Positioning of Longitudinal Axon Pathways
... neutralizing effect on the Netrin-mediated attraction is responsible for the longitudinal tracts entering the midline in slit mutants as opposed to a loss of repulsion as is currently ...This effect is not ...
12
Identification of a human synaptotagmin 1 mutation that perturbs synaptic vesicle cycling
... this dominant negative variant disturbs the dynamic properties of synaptic transmission, severely limiting postnatal functional brain organization, as indexed by infant-like auditory evoked ...
10
Critical roles of c Jun signaling in regulation of NFAT family and RANKL regulated osteoclast differentiation
... Nonstandard abbreviations used: activating transcription factor-2 (ATF-2); activa- tor protein-1 (AP-1); alkaline phosphatase (ALP); bone marrow macrophage (BMMΦ); bone morphogenetic protein-2 (BMP2); c-Jun N-terminal ...
11
Transactivation by the p65 subunit of NF kappaB in response to interleukin 1 (IL 1) involves MyD88, IL 1 receptor associated kinase 1, TRAF 6, and Rac1
... either dominant negative IRAK-1 or TRAF-6 completely inhibited the ability of MyD88 to transactivate gene ...addition, dominant negative MyD88 inhibited the effect of wild-type IRAK-1- ...
9