Macular crystalline inclusions in Sjögren-Larsson syndrome are dynamic structures that undergo remodeling

In an effort to understand the complex relationship between structure and biological function within the nucleus, we have embarked on a program to examine the three-dimensional structure and organization of Drosophila melanogaster embryonic chromosomes. Our overall goal is to determine how DNA and proteins are organized into complex and highly dynamic structures (chromosomes) and how these chromosomes are arranged in three dimensional space within the cell nucleus. Futher, we hope to be able to correlate structual data with such fundamental biological properties as stage in the mitotic cell cycle, developmental state and transcription at specific gene loci.Towards this end, we have been developing methodologies for the three-dimensional analysis of non-crystalline biological specimens using optical and electron microscopy. We feel that the combination of these two complementary techniques allows an unprecedented look at the structural organization of cellular components ranging in size from 100A to 100 microns.

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Muscle Tone Assessment under General Anesthesia for Sjögren-Larsson Syndrome and Spasticity

Pediatric Neurosurgery ◽

10.1159/000514329 ◽

2021 ◽

Vol 56 (2) ◽

pp. 163-165

Author(s):

George Georgoulis ◽

Argyrios Dinopoulos ◽

Emmanouil Gkliatis

Keyword(s):

General Anesthesia ◽

Muscle Tone ◽

Muscle Relaxants ◽

Spastic Diplegia ◽

Case Presentation ◽

Neurosurgical Procedure ◽

Dorsal Rhizotomy ◽

Severe Spasticity ◽

Larsson Syndrome ◽

Rule Out

Introduction: Study of muscle tone in individuals with severe spasticity (Modified Asworth Scale – MAS:3) under general anesthesia can confirm or rule out the eventual necessity of the impending spasticity relieving ablative neurosurgery by observing the hypertonia reduction and passive range of motion expansion. Therefore, what we measure under muscle relaxants is practically a fixed deformity. Case Presentation: The study was performed on a girl with Sjögren-Larsson syndrome, presenting with icthyosis and spastic diplegia. Proposed intervention was Dorsal Rhizotomy. Under general anesthesia, with and without muscle relaxants, hypertonia was significantly reduced (MAS:1), but the angle of motion did not increase much. Conclusion: We decided not to perform such a neurosurgical procedure. In ambiguous situations, the proposed study can help in decision-making for spasticity treatment.

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Monitoring modal shape of miniaturized dynamic structures via laser triangulation and stroboscopy

Microsystem Technologies ◽

10.1007/s00542-020-05159-z ◽

2021 ◽

Author(s):

Y. Emre Erdem ◽

Mustafa Berke Yelten ◽

Onur Ferhanoğlu

Keyword(s):

Laser Triangulation ◽

Modal Shape ◽

Dynamic Structures

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Recyclable Self-Healing Polyurethane Cross-Linked by Alkyl Diselenide with Enhanced Mechanical Properties

Polymers ◽

10.3390/polym11050773 ◽

2019 ◽

Vol 11 (5) ◽

pp. 773 ◽

Cited By ~ 3

Author(s):

Yuqing Qian ◽

Xiaowei An ◽

Xiaofei Huang ◽

Xiangqiang Pan ◽

Jian Zhu ◽

...

Keyword(s):

Mechanical Properties ◽

Room Temperature ◽

Amide Bond ◽

Self Healing ◽

Healing Efficiency ◽

Polyurethane Networks ◽

Combined Action ◽

Cross Linker ◽

Dynamic Structures ◽

External Interventions

Dynamic structures containing polymers can behave as thermosets at room temperature while maintaining good mechanical properties, showing good reprocessability, repairability, and recyclability. In this work, alkyl diselenide is effectively used as a dynamic cross-linker for the design of self-healing poly(urea–urethane) elastomers, which show quantitative healing efficiency at room temperature, without the need for any catalysts or external interventions. Due to the combined action of the urea bond and amide bond, the material has better mechanical properties. We also compared the self-healing effect of alkyl diselenide-based polyurethanes and alkyl disulfide-based polyurethanes. The alkyl diselenide has been incorporated into polyurethane networks using a para-substituted amine diphenyl alkyl diselenide. The resulting materials not only exhibit faster self-healing properties than the corresponding disulfide-based materials, but also show the ability to be processed at temperatures as low as 60 °C.

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Bezafibrate induces FALDH in human fibroblasts; implications for Sjögren-Larsson syndrome

Molecular Genetics and Metabolism ◽

10.1016/j.ymgme.2006.05.009 ◽

2006 ◽

Vol 89 (1-2) ◽

pp. 111-115 ◽

Cited By ~ 24

Author(s):

J GLOERICH ◽

L IJLST ◽

R WANDERS ◽

S FERDINANDUSSE

Keyword(s):

Human Fibroblasts ◽

Larsson Syndrome

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Prominence Seismology

Proceedings of the International Astronomical Union ◽

10.1017/s1743921313010703 ◽

2013 ◽

Vol 8 (S300) ◽

pp. 30-39 ◽

Cited By ~ 8

Author(s):

J. L. Ballester

Keyword(s):

Solar Corona ◽

Small Amplitude ◽

Dense Plasma ◽

Mhd Waves ◽

Physical Parameters ◽

Solar Prominences ◽

Dynamic Structures

AbstractQuiescent solar prominences are cool and dense plasma clouds located inside the hot and less dense solar corona. They are highly dynamic structures displaying flows, instabilities, oscillatory motions, etc. The oscillations have been mostly interpreted in terms of magnetohydrodynamic (MHD) waves, which has allowed to perform prominence seismology as a tool to determine prominence physical parameters difficult to measure. Here, several prominence seismology applications to large and small amplitude oscillations are reviewed.

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