Although the amygdalae play a central role in threat perception and reactions, the direct contributions of the amygdalae to specific aspects of threat perception, from ambiguity resolution to reflexive or deliberate action, remain ill understood in humans. Animal studies show that a detailed understanding requires a focus on the different subnuclei, which is not yet achieved in human research. ...

Lipoid proteinosis is a rare autosomal recessive disorder which may be seen within a family very occasionally. Herein, we report lipoid proteinosis in two sisters characterized by verrucous lesions and hoarseness of voice, dysphagia and multiple beaded papules along the margins of their eyelids, fissured lips and thick ferenulum.

Extracellular matrix protein 1 (ECM1) is expressed in a wide variety of tissues and plays important roles in extracellular matrix formation. Additionally, ECM1 gene mutations cause lipoid proteinosis (LP), a rare skin condition of genetic origin. However, an effective therapeutic approach of LP is not established. Here, we showed that ECM1 gene mutation observed in LP patients significantly sup...

Lipoid proteinosis is a very rare disorder in which there is infiltration of an amorphous hyaline material into the skin, oral cavity, larynx and various internal organs. It usually presents in infancy with hoarseness due to laryngeal infiltration. These patients usually develop acneiform or pock-like scars on the face either spontaneously or due to trauma. In this article, we describe a 27 yea...

Lipoid proteinosis (LP) is a rare disorder and it can affect every organ in the body. The clinical manifestations of LP may vary considerably between affected individuals. Short stature is reported in patients with LP however the underlying etiology is not clear. Short stature may be due to endocrine dysfunction caused by deposition of hyaline-like material in endocrine glands. We investigated ...

Lipoid proteinosis is a rare, autosomal-recessive, genetic disorder characterized by multisystem involvement due to intracellular deposition of amorphous hyaline material. The disease is due to a mutation in the extracellular matrix of the protein 1 gene. The skin, mucosa, and central nervous system are commonly affected. Hallmark findings in the brain are calcifications, mostly occurring in th...

Ruud Hortensius , David Terburg , Barak Morgan , Dan J. Stein , Jack van Honk b,c,g and Beatrice de Gelder a,b,* a Brain and Emotion Laboratory, Department of Cognitive Neuroscience, Faculty of Psychology and Neuroscience, Maastricht University, Maastricht, The Netherlands b Department of Psychiatry and Mental Health, University of Cape Town, J-Block, Groote Schuur Hospital, Cape Town, South Af...

Introduction Fear is an unpleasant emotional response to perceiving a threat causing physiological changes. Humans feel fear for positive motives, as it plays crucial role in our survival. Just the right balance life ideal, pathological often described one of its exaggerations, having too much. However, lack or “hypophobia” can be just devastating and debilitating. This demonstrated analogy bet...

The Urbach tail in crystalline GaAs and Si due to thermal vibration is evaluated in the temperature range 0—810 K. Recent experiments suggest that there is no thermally induced Urbach tail in undoped GaAs. This is most unexpected since thermally induced tails at the absorption edge having the Urbach form and widths Eo(T) linear in T are observed in a wide range of crystalline and amorphous semi...

We study a specific type of lifetime broadening resulting in the well-known exponential "Urbach tail" density of states within the energy gap of an insulator. After establishing the frequency and temperature dependence of the Urbach edge in GaAs quantum wells, we show that the broadening due to the zero-point optical phonons is the fundamental limit to the Urbach slope in high-quality samples. ...