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The protein encoded by IKBKAP is a scaffold protein and a regulator for 3 different kinases involved in proinflammatory signaling.
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Human Polyclonal IKBKAP Primary Antibody für ICC, ELISA - ABIN1002511
Krappmann, Hatada, Tegethoff, Li, Klippel, Giese, Baeuerle, Scheidereit: The I kappa B kinase (IKK) complex is tripartite and contains IKK gamma but not IKAP as a regular component. in The Journal of biological chemistry 2000
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Human Monoclonal IKBKAP Primary Antibody für IHC (p), ELISA - ABIN563666
Lefler, Cohen, Kantor, Cheishvili, Even, Birger, Turetsky, Gil, Even-Ram, Aizenman, Bashir, Maayan, Razin, Reubinoff, Weil: Familial Dysautonomia (FD) Human Embryonic Stem Cell Derived PNS Neurons Reveal that Synaptic Vesicular and Neuronal Transport Genes Are Directly or Indirectly Affected by IKBKAP Downregulation. in PLoS ONE 2015
show that the Elongator (elo1, elo2, and elo3) complex was purified from plant cell cultures as a six-component complex.
Elongator is important for plants to respond to ABA and drought exposure and that ABO1/ELO2 may play a vital role in ABA signal transduction pathways.[ABO1]
data reveal an essential function for Ikbkap that extends beyond the peripheral nervous system to CNS development and function
Results show that although Ikbkap is not required for normal development of retinal ganglion cells, its loss causes a slow, progressive retinal ganglion cells degeneration most severely in the temporal retina, which is later followed by indirect photoreceptor loss and complete retinal disorganization.
Our results show that tissue-specific expression of RBM24 (zeige RBM24 Antikörper) can explain the neuron-specific aberrant splicing of IKBKAP exon 20 in familial dysautonomia, and that ectopic expression of RBM24 (zeige RBM24 Antikörper) in neuronal tissue could be a novel therapeutic target of the disease.
ELP1 has a neuron autonomous role in sympathetic and sensory target tissue innervation
Phosphatidylserine increases IKBKAP levels in a humanized knock-in IKBKAP mouse model for Familial dysautonomia.
Familial dysautonomia model reveals Ikbkap deletion causes apoptosis of Pax3 (zeige PAX3 Antikörper)+ progenitors and peripheral neurons.
our study not only reveals a novel function of IKAP in meiosis, but also suggests that IKAP contributes to this process partly by exerting its effect on transcription and tRNA modification
IKAP expression levels modulate disease severity in a mouse model of familial dysautonomia.
IKAP is essential for expression of specific genes involved in cardiac morphogenesis (zeige XIRP1 Antikörper), and cardiac failure is the likely cause of abnormal vascular development and embryonic lethality; deletion of exon 20 abolishes gene function
mutations in the human I-kappaB kinase-associated protein (IKBKAP) gene are responsible for familial dysautonomia (FD).no animal model of FD currently exists, cloning of the mouse Ikbkap gene is an important step toward creating a mouse model for FD
overexpression of miR (zeige MLXIP Antikörper)-203a-3p leads to a decrease of NOVA1 (zeige NOVA1 Antikörper), counter-balanced by an increase of IKAP, supporting a potential interaction between NOVA1 (zeige NOVA1 Antikörper) and IKAP.
IKAP might be a vesicular like protein that might be involved in neuronal transport in hESC derived PNS neurons
The formation of the Elp1 dimer contributes to its stability in vitro and in vivo and is required for the assembly of human Elongator complexes.
IKBKAP mRNA levels decreased during a familial dysautonomia crisis and returned to baseline after recovery. The cause-and-effect relationship is unclear.
Digoxin-mediated repression of SRSF3 (zeige SRSF3 Antikörper) expression plays a role in the digoxin-mediated inclusion of exon 20 in the IKBKAP transcript generated from the familial dysautonomia mutant allele.
Combined treatment with epigallocatechin gallate and genistein synergistically upregulates wild-type IKBKAP-encoded RNA and protein levels in familial dysautonomia-derived cells.
IKAP plays pleiotropic roles in both the peripheral and central nervous systems
IKAP/hELP1 deficiency has an effect on gene expression in differentiating neuroblastoma (zeige ARHGEF16 Antikörper) cells, and possibly on familial dysautonomia
IKK complex-associated protein deficiency upregulates the microtubule destabilizing protein SCG10 (zeige STMN2 Antikörper) and, in parallel, disorganizes the cytoskeleton
The protein encoded by this gene is a scaffold protein and a regulator for 3 different kinases involved in proinflammatory signaling. This encoded protein can bind NF-kappa-B-inducing kinase (NIK) and IKKs through separate domains and assemble them into an active kinase complex. Mutations in this gene have been associated with familial dysautonomia.
elongator complex protein 1
, inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase complex-associated protein
, IKK complex-associated protein
, IKK-complex-associated protein IKAP
, ikappaB kinase complex-associated protein
, inhibitor of kappa light polypeptide enhancer in B-cells, kinase complex-associated protein
, elongator acetyltransferase complex subunit 1
, putative elongator complex protein 1