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            : Lipoid proteinosis (Urbach-Wiethe disease)

Quirici, Marcelo Bianco; Rocha, Antônio José da

doi:10.1212/wnl.0b013e3182840741

Cited by 6 publications

(3 citation statements)

References 2 publications

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“…Five female participants with Urbach-Wiethe disease (UWD) partly from the South African UWD cohort [51] and 12 matched controls took part in the experiment. UWD, also known as lipoid proteinosis [52], is a rare genetic disorder that leads to a variety of symptoms such as thickening of the skin and beaded eyelid papules, and also results in calcification of brain tissue. Mutations in the extracellular matrix protein (ECM1) gene on chromosome 1 (1q21) are responsible for UWD symptoms [53].…”

Section: Materials and Methods (A) Participantsmentioning

confidence: 99%

The role of the basolateral amygdala in the perception of faces in natural contexts

Hortensius

Terburg

Morgan

et al. 2016

Phil. Trans. R. Soc. B

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One contribution of 15 to a theme issue 'Attending to and neglecting people'. The amygdala is a complex structure that plays its role in perception and threatrelated behaviour by activity of its specific nuclei and their separate networks. In the present functional magnetic resonance imaging study, we investigated the role of the basolateral amygdala in face and context processing. Five individuals with focal basolateral amygdala damage and 12 matched controls viewed fearful or neutral faces in a threatening or neutral context. We tested the hypothesis that basolateral amygdala damage modifies the relation between face and threatening context, triggering threat-related activation in the dorsal stream. The findings supported this hypothesis. First, activation was increased in the right precentral gyrus for threatening versus neutral scenes in the basolateral amygdala damage group compared with the control group. Second, activity in the bilateral middle frontal gyrus, and left anterior inferior parietal lobule was enhanced for neutral faces presented in a threatening versus neutral scene in the group with basolateral amygdala damage compared with controls. These findings provide the first evidence for the neural consequences of basolateral amygdala damage during the processing of complex emotional situations.

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Section: Materials and Methods (A) Participantsmentioning

confidence: 99%

The role of the basolateral amygdala in the perception of faces in natural contexts

Hortensius

Terburg

Morgan

et al. 2016

Phil. Trans. R. Soc. B

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“…Extracellular matrix protein 1 (ECM1) is a multifunctional protein involved in the regulation of ureteric bud patterning and branching (2), lung regeneration in post‐pneumonectomy mice (3), and various disease processes, including lipoid proteinosis (4–6), lichen sclerosus (7–10), and ulcerative colitis (11, 12). ECM1 plays a critical role in the acquisition of immune tolerance and allergic responses via particular T‐cell subsets, such as CD4 + CD25 + regulatory T cells and Th2 cells (13).…”

mentioning

confidence: 99%

Extracellular matrix protein 1, a direct targeting molecule of parathyroid hormone‐related peptide, negatively regulates chondrogenesis and endochondral ossificationviaassociating with progranulin growth factor

et al. 2016

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Chondrogenesis and endochondral ossification are precisely controlled by cellular interactions with surrounding matrix proteins and growth factors that mediate cellular signaling pathways. Here, we report that extracellular matrix protein 1 (ECM1) is a previously unrecognized regulator of chondrogenesis. ECM1 is induced in the course of chondrogenesis and its expression in chondrocytes strictly depends on parathyroid hormone-related peptide (PTHrP) signaling pathway. Overexpression of ECM1 suppresses, whereas suppression of ECM1 enhances, chondrocyte differentiation and hypertrophy in vitro and ex vivo. In addition, target transgene of ECM1 in chondrocytes or osteoblasts in mice leads to striking defects in cartilage development and endochondral bone formation. Of importance, ECM1 seems to be critical for PTHrP action in chondrogenesis, as blockage of ECM1 nearly abolishes PTHrP regulation of chondrocyte hypertrophy, and overexpression of ECM1 rescues disorganized growth plates of PTHrP-null mice. Furthermore, ECM1 and progranulin chondrogenic growth factor constitute an interaction network and act in concert in the regulation of

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“…UrbacheWiethe Disease (UWD) provides the neuroscience community with a unique possibility to study the functions of the amygdala in a causal way. UWD or lipoid proteinosis is an extremely rare autosomal recessive genetic disease that leads to thickening of the skin, beaded eyelid papules, other dermatological symptoms and calcification of brain tissue (Quirici & da Rocha, 2013). So far, 250 to 300 cases have been described in the literature, with current estimations of less than 100 individuals alive (van Honk, Terburg, Thornton, Stein, & Morgan, 2016).…”

mentioning

confidence: 99%

The dynamic consequences of amygdala damage on threat processing in Urbach–Wiethe Disease. A commentary on Pishnamazi et al. (2016)

Hortensius

Terburg

Morgan

et al. 2017

Cortex

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Teaching Neuro Images : Lipoid proteinosis (Urbach-Wiethe disease)

Cited by 6 publications

References 2 publications

The role of the basolateral amygdala in the perception of faces in natural contexts

The role of the basolateral amygdala in the perception of faces in natural contexts

Extracellular matrix protein 1, a direct targeting molecule of parathyroid hormone‐related peptide, negatively regulates chondrogenesis and endochondral ossificationviaassociating with progranulin growth factor

The dynamic consequences of amygdala damage on threat processing in Urbach–Wiethe Disease. A commentary on Pishnamazi et al. (2016)

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