Disease relevance of TNFRSF11A

• Mutations in TNFRSF11A, affecting the signal peptide of RANK, cause familial expansile osteolysis [1].
• We identified two heterozygous insertion mutations in exon 1 of TNFRSF11A in affected members of four families with FEO or familial Paget disease of bone (PDB) [1].
• These data indicate that TNFRSF11A mutations contribute neither to the vast majority of cases of sporadic or familial PDB, nor to the development of osteosarcoma [2].
• Malignant autosomal recessive osteopetrosis caused by spontaneous mutation of murine Rank [3].
• Interestingly, a significant correlation (r = 0.73, p < 0.0001, Spearman Rank test) was noted between MBHR and intensity of bronchoalveolar lavage eosinophilia at 48 hours [4].

Psychiatry related information on TNFRSF11A

• The author traces the historical background of brief psychotherapy, focusing on the contributions of Freud, Ferenczi, Rank, and Alexander and French, and then presents a synthesis of contemporary views [5].
• There were no differences between the first two groups with respect to the distribution of PSE symptoms and signs, Schneider's First Rank Symptoms, or severity of psychopathology [6].
• The cooperative term, which amounts to only about 10% of the total hydrophobic association free energy, was found to increase the strength of hydrophobic association; this finding differs from the results of earlier Monte Carlo studies with the free energy perturbation method of Rank and Baker (1997) [7].
• Among persons who do not report risk behaviors for HIV infection, men had substantially higher median rates of HIV infection than women (P less than 0.001, Wilcoxon Signed Rank test), and rates were positively correlated with HIV infection rates in IV drug users in the same clinic (Pearson correlation coefficient [r] = 0.8; P less than 0.001) [8].
• We rated spoken language (Spoken Language Rank, SLR) on a scale from 0 (no language) to 6 (mature grammar) and examined the effects of side of resection/damage, age at surgery/seizure onset, seizure control postsurgery, and etiology on language development [9].

High impact information on TNFRSF11A

• Our previous linkage studies mapped the gene responsible for FEO to an interval of less than 5 cM between D18S64 and D18S51 on chromosome 18q21.2-21.3 in a large Northern Irish family [1].
• TRANCE, a TNF family member, and its receptor, TRANCE-R, are critical regulators of dendritic cell and osteoclast function [10].
• Thus, TRANCE expression by osteoblasts appears to be both necessary and sufficient for hormone-mediated activation of mature osteoclasts, and TRANCE-R is likely to be a receptor for signal transduction for activation of the osteoclast and its survival [11].
• Here, we show that high levels of TRANCE-R are detected on mature dendritic cells (DCs) but not on freshly isolated B cells, T cells, or macrophages [12].
• In this report we identify the TNF receptor-associated factor (TRAF) family of signal transducers as important components of TRANCE-R-mediated NF-kappaB activation [13].

Chemical compound and disease context of TNFRSF11A

• Ionization of the surface constituents of Edwardsiella tarda cells provided efficient surface charges for the intact cells to be separated from the EOF and damaged or lysed cells when the separation was performed in running buffer (3.94 mM Tris, 0.56 mM borate, 0.013 mM EDTA) at pH 10 [14].
• Among psychoanalysts of all colorations respecting their Freudian orthodoxy, there is a special mystique and nostalgia around the Oedipus complex and paramountcy of the father in a child's mental life; but Otto Rank took a militant, yet reasoned, stand against such patriarchal biases [15].

Biological context of TNFRSF11A

• Mutational analysis of the TNFRSF11A gene in our patients demonstrated an 18 base pair tandem duplication, one base proximal to the duplications previously reported [16].
• The TNFRSF11A gene encodes the receptor activator of nuclear factor-kappaB (RANK), which has been demonstrated to be essential in bone remodeling and osteoclast differentiation [16].
• Non-parametric sibling pair linkage analysis identified evidence of linkage (LOD = 2.5; p = 0.02) of EARR affecting the maxillary central incisor with the microsatellite marker D18S64 (tightly linked to TNFRSF11A) [17].
• Furthermore, transfection experiments with TRANCE-R deletion mutants revealed that multiple regions of the TRANCE-R can mediate NF-kappaB activation [13].
• Loss of heterozygosity (LOH) affecting the PDB/FEO critical region has also been described in osteosarcomas suggesting that TNFRSF11A might also be involved in the development of osteosarcoma [2].

Anatomical context of TNFRSF11A

• No specific abnormalities of the TNFRSF11A gene were identified in a Pagetic osteosarcoma, the osteosarcoma cell lines, DNA extracted from Pagetic bone lesions, or DNA extracted from peripheral blood in patients with familial or sporadic Paget's disease including several individuals with early onset Paget's disease [2].
• CONCLUSIONS: The phenotypic similarities between Rank(del8) and mice previously described with a combined insertion and deletion in Rank confirm the role of this receptor in osteoclastogenesis and lymph node development and suggest that some forms of malignant osteopetrosis in humans could result from a similar defect [3].
• To understand the ODF-mediated signal transduction mechanism in osteoclastogenesis, we molecularly cloned ODFR from a mouse macrophage-like osteoclast progenitor cell line, C7 [18].
• In coated capillaries where the EOF is suppressed, however, our model predicts a lower plate number in the presence of an initial voltage ramp [19].
• We found that nonionic surfactants worked best: they lyse the plasma membrane without significantly influencing K(d), k(off), or the EOF [20].

Associations of TNFRSF11A with chemical compounds

• Mitochondrial respiration was determined using a Rank oxygen polarograph at 37 degrees C in a sucrose and mannitol respiration medium [21].
• There was a strong positive relationship between MIB-1 and BUDR by Spearman Rank correlation [22].
• We report an online concentration and separation method for basic proteins using poly(diallyldimethylammonium chloride) (PDDA) solutions in the presence of reversed EOF [23].
• With zwitterionic additives of the form +NH3-(CH2)n-COO-, the magnitude of the EOF increase is directly related to the number of methylene groups, (n), which ranges from n = 1 to 7 [24].
• Our developed method effectively utilized the reversed flow from this cathodic EOF as a driving force for sample proteins using permanently uncoated but dynamic SDS-coated PMMA microchips [25].

Other interactions of TNFRSF11A

• This new region is approximately 500 kb and contains at least 7 known genes; however, it excludes 2 previous candidate genes: TNFRSF11A (RANK) and BCL2 [26].
• Using a candidate gene approach, we investigated possible linkage of EARR associated with orthodontic treatment with the TNSALP, TNFalpha, and TNFRSF11A gene loci [17].
• Our results suggest that differences in the osteoclastogenesis-inducing capacity of TRANCE-R versus other TRAF6-associated receptors may in part stem from a quantitative difference in the TRAF6-mediated signals [27].
• IL-6 was increased in children after TBI versus controls on all days studied (day 1, 3158.2 +/- 621.8 pg/ml; day 2, 1111.6 +/- 337.0 pg/ml; day 3, 826.7 +/- 193.5 pg/ml vs. 20.6 +/- 5.8 pg/ml, p < 0.0001, Mann-Whitney Rank Sum) [28].
• Insertion mutations of the TNFRSF11A gene encoding Receptor Activator of NF kappa B (RANK) have recently been found to be responsible for FEO and rare cases of early onset familial Paget's disease [2].

Analytical, diagnostic and therapeutic context of TNFRSF11A

• Manipulation of the EOF is essential for achieving optimal separations by MEKC [29].
• This article presents a 3-D mathematical model for Joule heating and its effects on the EOF and electrophoretic transport of solutes in microfluidic channels [30].
• The microparticle image velocimetry technique was used to measure the velocity profiles of EOF under the influence of Joule heating [30].
• The high EOF generated by the coatings facilitated the maintenance of a stable spray when coupling to ESI-MS, and a stable CZE current when working with a sheath flow-assisted analyte deposition onto MALDI targets, respectively [31].
• The new copolymers, due to their low viscosity high sieving capacity and ability to suppress EOF, represent a better alternative to PDMA and are suitable replaceable matrices for capillary and microchip electrophoresis [32].

References