Management of the dentoalveolar effects of tongue hypertrophy in patients with Duchenne muscular dystrophy: A pilot study.

Subjects affected by Duchenne muscular dystrophy (DMD) develop severe malocclusions with the progression of the disease, due to changes in orofacial musculature and function, including tongue hypertrophy. We aimed to evaluate the benefits of maintaining mandibular interarch width with the help of a simple fixed orthodontic appliance. Three adolescent DMD boys were selected consecutively to receive a passive rigid mandibular lingual arch, and followed for 4-5 years.

Growing patients with Duchenne muscular dystrophy: longitudinal changes in their dentofacial morphology and orofacial functional capacities.

Aim: The aim of this study was to describe the longitudinal changes in facial morphology, dental arch alterations and oral functional capacities that occur in growing patients with Duchenne muscular dystrophy (DMD) in order to identify the effects of the progression of the disease.

Subjects And Methods: Twelve DMD patients (6.5-17.

Plasmid-like replication of a minimal streptococcal integrative and conjugative element.

Integrative and conjugative elements (ICEs) are mobile genetic elements encoding their own excision from a replicon of their bacterial host, transfer by conjugation to a recipient bacterium and reintegration for maintenance. The conjugation, recombination and regulation modules of ICEs of the ICE family are grouped together in a region called the ICE 'core region'. In addition to this core region, elements belonging to this family carry a highly variable region including cargo genes that could be involved in bacterial adaptation or in the maintenance of the element.

Differential regulation of two closely related integrative and conjugative elements from Streptococcus thermophilus.

Background: Two closely related ICEs, ICESt1 and ICESt3, have been identified in the lactic acid bacterium Streptococcus thermophilus. While their conjugation and recombination modules are almost identical (95% nucleotide identity) and their regulation modules related, previous work has demonstrated that transconjugants carrying ICESt3 were generated at rate exceeding by a 1000 factor that of ICESt1.

Results: The functional regulation of ICESt1 and ICESt3 transcription, excision and replication were investigated under different conditions (exponential growth or stationary phase, DNA damage by exposition to mitomycin C).

Site-specific accretion of an integrative conjugative element together with a related genomic island leads to cis mobilization and gene capture.

Genomic islands, flanked by attachment sites, devoid of conjugation and recombination modules and related to the integrative and conjugative element (ICE) ICESt3, were previously found in Streptococcus thermophilus. Here, we show that ICESt3 transfers to a recipient harbouring a similar engineered genomic island, CIMEL₃catR₃, and integrates by site-specific recombination into its attachment sites, leading to their accretion. The resulting composite island can excise, showing that ICESt3 mobilizes CIMEL₃catR₃, in cis.

Conjugative transfer of the integrative conjugative elements ICESt1 and ICESt3 from Streptococcus thermophilus.

Integrative and conjugative elements (ICEs), also called conjugative transposons, are genomic islands that excise, self-transfer by conjugation, and integrate in the genome of the recipient bacterium. The current investigation shows the intraspecies conjugative transfer of the first described ICEs in Streptococcus thermophilus, ICESt1 and ICESt3. Mitomycin C, a DNA-damaging agent, derepresses ICESt3 conjugative transfer almost 25-fold.

Regulation of excision of integrative and potentially conjugative elements from Streptococcus thermophilus: role of the arp1 repressor.

The integrative and conjugative elements (ICEs) excise by site-specific recombination between attL and attR flanking sites, self-transfer the resulting circular form and integrate into the genome of the recipient cell. Two putative ICEs, ICESt1 and ICESt3, are integrated in the same locus in 2 strains of Streptococcusthermophilus. ICESt1 is a composite element harbouring an internal recombination site, attL'.

Derepression of excision of integrative and potentially conjugative elements from Streptococcus thermophilus by DNA damage response: implication of a cI-related repressor.

A DNA-damaging agent, mitomycin C, derepresses the site-specific excision of two integrative and potentially conjugative elements from Streptococcus thermophilus, ICESt1 and ICESt3. The regulation pathway involves a repressor related to phage lambda cI repressor. It could also involve a putative regulator related to another type of phage repressors, the "cI-like" repressors.

The constant gene orf14.9, which belongs to the variable eps (exopolysaccharide) cluster, is involved in the cell growth of Streptococcus thermophilus.

In Streptococcus thermophilus, the eps clusters involved in exopolysaccharide (EPS) biosynthesis are very polymorphic, nevertheless they all contain a highly conserved sequence corresponding to that of orf14.9. This open reading frame (ORF) is transcribed in a reverse direction with respect to eps genes.

Increased Wnt signaling triggers oncogenic conversion of human breast epithelial cells by a Notch-dependent mechanism.

Wnt and Notch signaling have long been established as strongly oncogenic in the mouse mammary gland. Aberrant expression of several Wnts and other components of this pathway in human breast carcinomas has been reported, but evidence for a causative role in the human disease has been missing. Here we report that increased Wnt signaling, as achieved by ectopic expression of Wnt-1, triggers the DNA damage response (DDR) and an ensuing cascade of events resulting in tumorigenic conversion of primary human mammary epithelial cells.

Partial rhombencephalosynapsis.

We describe an infant in whom partial rhombencephalosynapsis was diagnosed by using MR imaging. The anterior vermis and nodulus were normally developed, but part of the posterior vermis was deficient. There was partial fusion of the hemispheres in the inferior part of the cerebellum.