A large cellulosomal gene cluster was identified in the recently sequenced genome of Clostridium acetobutylicum ATCC 824. Sequence analysis revealed that this cluster contains the genes for the scaffolding protein CipA, the processive endocellulase Cel48A, several endoglucanases of families 5 and 9, the mannanase Man5G, and a hydrophobic protein, OrfXp. Surprisingly, genetic organization of thi...

BACKGROUND Congenital insensitivity to pain with anhidrosis is an extremely rare disorder in which injuries can often be missed by patient, parents and even by orthopedic surgeon. Pain and tenderness, on which a trauma team so much depends to make a clinical diagnosis and to decide whether to go for radiological evaluation can be misleading in this rare syndrome. So complete clinical examinatio...

Hereditary sensory and autonomic neuropathy type IV is a rare disease characterized by fever episodes, mental retardation of different intensity, recurrent episodes of fever secondary to anhidrosis, little or no perspiration and congenital insensitivity to pain. Oral self-mutilation is also a characteristic sign. In this article, we present the case of an infant, aged 22 months, who showed thes...

Congenital insensitivity to pain with anhydrosis (CIPA) is a rare genetic disorder characterized by inability to feel pain and temperature, and decreased or absent sweating. Familiarity to this condition is important to avoid misdiagnosing it with leprosy and other peripheral nerve diseases. An inability to feel pain may lead to repeated self-trauma (tongue, lips and finger tips) ultimately lea...

According to the emotion-context insensitivity (ECI) hypothesis, major depressive disorder (MDD) is associated with a diminished ability to react emotionally to positive stimuli and with blunting of defensive responses to threat. That defensive responses are blunted in MDD seems inconsistent with the conceptualization and diagnostic nosology of MDD. The present study tested the ECI hypothesis i...

Classical methodologies used in data collection for architectural documentation are studied (surveying topographic, photogrammetry, manual methods, etc). Different aspects are analysed in order to select the most suitable approach, always taking into account the specific determining factors. The suggested technique is in line with the so-called close range photogrammetry by simple methods. Its ...

Clostridium thermocellum produces an extracellular multienzyme complex, termed the cellulosome, that allows efficient solubilization of crystalline cellulose. The complex is organized around a large noncatalytic protein subunit, termed CipA or scaffoldin, and is found either free in the supernatant or cell bound. The binding of the complex to the cell is mediated by three cell surface anchoring...

Congenital Insensitivity to pain with anhydrosis (CIPA) is a rare inherited disease. It is classified as hereditary sensory and autonomic neuropathy type IV. Pain insensitivity and autonomic deficits are present, but touch and pressure sensitivity are unimpaired. Mental retardation is usually present. We report a family case of a 5 years old girl and 2 years old boy with congenital insensitivit...

L'insensibilité congénitale à la douleur (ICD), est une affection rare de transmission autosomique récessive, caractérisée par une absence congénitale de la perception de la douleur. Elle a été décrite pour la première fois en 1932 par DEARBORN, elle constitue le type V des neuropathies héréditaires sensitives et autonomiques (HSAN :hereditary sensory and autonomic neuropathies). Cette patholog...

Stochastic activity networks (SANs) are a powerful and flexible extension of Petri nets. These models can be used for the modeling and analysis of various kinds and different aspects of distributed real-time systems. Similar to other classical extensions of Petri nets, SANs have some limitations for modeling complex and largescale systems. In order to remove these limitations and provide some h...