Artemis, DNA ligase IV, DNA protein kinase catalytic subunit, and Cernunnos/XLF genes in nonhomologous end joining pathways of DNA repair mechanisms have been identified as responsible for radiosensitive SCID. Here, we present a 3-year-old girl patient with severe growth retardation, bird-like face, recurrent perianal abscess, pancytopenia, and polydactyly. Firstly, she was thought as Fanconi a...

Developing B and T lymphocytes generate programmed DNA double strand breaks (DSBs) during the V(D)J recombination process that assembles exons that encode the antigen-binding variable regions of antibodies. In addition, mature B lymphocytes generate programmed DSBs during the immunoglobulin heavy chain (IgH) class switch recombination (CSR) process that allows expression of different antibody h...

Using the population synthesis code StarTrack we construct the first synthetic X-ray binary populations for direct comparison with the X-ray luminosity function (XLF) of NGC 1569 observed with Chandra. Our main goal is to examine whether it is possible to reproduce the XLF shape with our models, given the current knowledge for the star-formation history of this starburst galaxy. We thus produce...

The double-strand DNA break repair pathway, non-homologous DNA end joining (NHEJ), is distinctive for the flexibility of its nuclease, polymerase and ligase activities. Here we find that the joining of ends by XRCC4-ligase IV is markedly influenced by the terminal sequence, and a steric hindrance model can account for this. XLF (Cernunnos) stimulates the joining of both incompatible DNA ends an...

IRAS 20050+2720 is young star forming region at a distance of 700 pc without apparent high mass stars. We present results of our multiwavelength study of IRAS 20050+2720 which includes observations by Chandra and Spitzer, and 2MASS and UBVRI photometry. In total, about 300 YSOs in different evolutionary stages are found. We characterize the distribution of young stellar objects (YSOs) in this r...

Risks embedded in asset price dynamics are taken to be accumulations of surprise jumps. AMarkov pure jumpmodel is formulated on making variance gamma parameters deterministic functions of the price level. Estimation is done by matrix exponentiation of the transition rate matrix for a continuous time finite state Markov chain approximation. The motion is decomposed into a space dependent drift a...

To ensure genome stability, mammalian cells employ several DNA repair pathways. Nonhomologous DNA end joining (NHEJ) is the DNA repair process that fixes double-strand breaks throughout the cell cycle. NHEJ is involved in the development of B and T lymphocytes through its function in V(D)J recombination and class switch recombination (CSR). NHEJ consists of several core and accessory factors, i...

We present the X-ray luminosity function (XLF) for clusters of galaxies derived from the RASS1 Bright Sample. The sample, selected from the ROSAT All-Sky Survey in a region of 2.5 sr within the southern Galactic cap, contains 130 clusters with flux limits in the range ∼ 3− 4× 10 ergs cm s in the 0.5-2.0 keV band. A maximum-likelihood fit with a Schechter function of the XLF over the entire rang...

Nonhomologous end joining is the primary deoxyribonucleic acid (DNA) double-strand break repair pathway in multicellular eukaryotes. To initiate repair, Ku binds DNA ends and recruits the DNA-dependent protein kinase (DNA-PK) catalytic subunit (DNA-PKcs) forming the holoenzyme. Early end synapsis is associated with kinase autophosphorylation. The XRCC4 (X4)-DNA Ligase IV (LIG4) complex (X4LIG4)...

Eukaryotic cells use two principal mechanisms for repairing DNA double-strand breaks (DSBs): homologous recombination (HR) and nonhomologous end-joining (NHEJ). DSB repair pathway choice is strongly regulated during the cell cycle. Cyclin-dependent kinase 1 (Cdk1) activates HR by phosphorylation of key recombination factors. However, a mechanism for regulating the NHEJ pathway has not been esta...