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anti-Human BBS4 Anticorps:
anti-Rat (Rattus) BBS4 Anticorps:
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Human Monoclonal BBS4 Primary Antibody pour WB - ABIN1882046
Mykytyn, Braun, Carmi, Haider, Searby, Shastri, Beck, Wright, Iannaccone, Elbedour, Riise, Baldi, Raas-Rothschild, Gorman, Duhl, Jacobson, Casavant, Stone, Sheffield: Identification of the gene that, when mutated, causes the human obesity syndrome BBS4. dans Nature genetics 2001
Show all 5 Pubmed References
Human Polyclonal BBS4 Primary Antibody pour ELISA, WB - ABIN4283224
Shah, Farmen, Moninger, Businga, Andrews, Bugge, Searby, Nishimura, Brogden, Kline, Sheffield, Welsh: Loss of Bardet-Biedl syndrome proteins alters the morphology and function of motile cilia in airway epithelia. dans Proceedings of the National Academy of Sciences of the United States of America 2008
we show that a centriolar satellite protein, AZI1 (Montrer AZI1 Anticorps) (also known as CEP131 (Montrer AZI1 Anticorps)), interacts with the BBSome and regulates BBSome ciliary trafficking activity. Furthermore, we show that AZI1 (Montrer AZI1 Anticorps) interacts with the BBSome through BBS4
loss of BBS1 (Montrer BBS1 Anticorps), BBS4, or OFD1 led to decreased NF-kappaB (Montrer NFKB1 Anticorps) activity and concomitant IkappaBbeta (Montrer NFKBIB Anticorps) accumulation and that these defects were ameliorated with SFN (Montrer SFN Anticorps) treatment.
a novel nonsense mutation in BBS4 gene in a Chinese family with Bardet-Biedl syndrome. This homozygous mutation was predicted to completely abolish the synthesis of the BBS4 protein; a rare heterozygous missense SNP in BBS10 (Montrer BBS10 Anticorps) gene was also detected
Results present evidence of a role for BBS4 in mediating the phosphorylation of TrkB (Montrer NTRK2 Anticorps) by BDNF (Montrer BDNF Anticorps) and its activation requires a proper localization to the ciliary axoneme.
mediates endosomal recycling, sorting and signal transduction of Notch (Montrer NOTCH1 Anticorps) receptors
loss of BBS1 (Montrer BBS1 Anticorps), BBS4, or OFD1 (Montrer OFD1 Anticorps) led to decreased NF-kappaB (Montrer NFKB1 Anticorps) activity and concomitant IkappaBbeta (Montrer NFKBIB Anticorps) accumulation and that these defects were ameliorated with SFN (Montrer SFN Anticorps) treatment.
Findings indicate that Bbs (Montrer BBS2 Anticorps) proteins play a central role in the regulation of the actin cytoskeleton and control the cilia length through alteration of RhoA (Montrer RHOA Anticorps) levels.
Ectopic expression of human BBS4 can rescue Bardet-Biedl syndrome phenotypes in Bbs4 null mice.
A novel missense mutation in BBS4 that co-segregates with Leber Congenital Amaurosis was identified in a consanguineous family from Saudi Arabia.
BBS4 is a minor contributor to Bardet-Biedl syndrome and may also participate in triallelic inheritance. Evaluated the spectrum of mutations in the recently identified BBS4 gene with a combination of haplotype analysis and mutation screening.
The phenotype of patients with BBS4 mutations consists of severe retinitis pigmentosa, variable obesity, brachydactyly with variable polydactyly, small or missing teeth, genital hypoplasia, and cardiovascular disease.
A novel Frameshift Mutation between the splice donor site and exon 5 of BBS4 in a Bardet-Biedl syndrome patient and a novel heterozygous base substitution in both an affected mother and her affected daughter.
demonstrated that the MKS (Montrer MKKS Anticorps) transition zone complex cooperates with the BBSome to mediate trafficking of specific trans-membrane receptors to the cilium
Bbs1 (Montrer BBS1 Anticorps), Bbs2 (Montrer BBS2 Anticorps), and Bbs4 proteins (BBSome) are bona fide constituents of intraflagellar transport in olfactory sensory neurons.
Bbs4 silencing in 3T3F442A preadipocytes induced accelerated cell division and aberrant differentiation. Bbs4 silenced cells accumulate significantly more triglycerides than control adipocytes.
The BBSome binds to the N-terminal region of CEP290 (Montrer CEP290 Anticorps) through BBS4 and co-localizes with CEP290 (Montrer CEP290 Anticorps) to the transition zone (TZ) of primary cilia and centriolar satellites in ciliated cells, as well as to the connecting cilium in photoreceptor cells.
Bbs4-null mice develop both motile and primary cilia, demonstrating that Bbs4 is not required for global cilia formation.
deletions of Bbs1 (Montrer BBS1 Anticorps) or Bbs4 affected the olfactory epithelium, causing severe reduction of the ciliated border, disorganization of the dendritic microtubule network and trapping of olfactory ciliary proteins in dendrites and cell bodies.
Evaluations of mice null for Bbs4, have uncovered phenotypic features with age-dependent penetrance and variable expressivity, partially recapitulating the human Bardet-Biedl syndrome phenotype.
Lacking Bbs4 does not lead to aberrant cilia or basal body structure. However, the dynamics of cilia assembly is altered in Bbs4(-/-) cells, suggesting a role for Bbs4 in the regulation of ciliary assembly.
The specific loss of photoreceptors in Bbs4(-)(/)(-) mice allows us to identify a set of genes that are preferentially expressed in photoreceptors compared with other cell types found in the eye
This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by severe pigmentary retinopathy, obesity, polydactyly, renal malformation and mental retardation. The proteins encoded by BBS gene family members are structurally diverse. The similar phenotypes exhibited by mutations in BBS gene family members are likely due to the protein's shared roles in cilia formation and function. Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells. BBS proteins may also be involved in intracellular trafficking via microtubule-related transport. The protein encoded by this gene has sequence similarity to O-linked N-acetylglucosamine (O-GlcNAc) transferases in plants and archaebacteria and in human forms a multi-protein 'BBSome' complex with six other BBS proteins. Alternative splice variants have been described but their predicted protein products have not been experimentally verified.
, Bardet-Biedl syndrome 4
, Bardet-Biedl syndrome 4 protein homolog
, bardet-biedl syndrome 4
, bardet-Biedl syndrome 4 protein-like
, Bardet-Biedl syndrome 4 protein-like
, Bardet-Biedl syndrome 4 protein
, Bardet-Biedl syndrome 4 homolog