Quadruple genetic variants in a sporadic ALS patient.

Objectives: Due to upcoming gene-specific therapy approaches for ALS patients, understanding familial and sporadic ALS genetics is becoming increasingly important. In this study, we wanted to investigate underlying genetic causes for an SALS patient.

Methods: We performed ALS gene panel sequencing and subsequent segregation analysis in the family.

Black and white fused silica: modified sol-gel process combined with moth-eye structuring for highly absorbing and diffuse reflecting SiO glass.

Diffuse reflecting (white) and highly absorbing (black) fused silica based materials are presented, which combine volume modified substrates and surfaces equipped with anti-reflective moth-eye-structures. For diffuse reflection, micrometer sized cavities are created in bulk fused silica during a sol-gel process. In contrast, carbon black particles are added to get the highly absorbing material.

Combined 'moth-eye' structured and graded index-layer anti-reflecting coating for high index glasses.

We present a hybrid antireflective coating (ARC) providing a complete continuous graded refractive index (GRIN) transition from a high-index substrate down to ambient air. The ARC comprises a first GRIN layer of dense silicon-oxy-nitride with a varying, height adjusted material composition. Secondly, a layer of quasi-periodic nanopillars imitating AR-"moth-eye structure" is added to the dense GRIN layer.

Heat release at the wetting front during capillary filling of cellulosic micro-substrates.

Spontaneous imbibition in cellulosic materials is an expanding field of research due to the direct applicability in paper-based microfluidics. Here, we show experimentally, using simultaneous thermal and optical imaging that the temperature at the wetting front during capillary filling of paper is temporarily increased, even if the imbibed fluid and the cellulosic substrate are initially at isothermal conditions. Several liquids and two types of filter paper, characterised by scanning electron microscopy (SEM) and X-ray diffraction (XRD) analysis, were investigated demonstrating a significant temperature rise at the wetting front that cannot be neglected form the process.

The role of the peripheral benzodiazepine receptor in photodynamic activity of certain pyropheophorbide ether photosensitizers: albumin site II as a surrogate marker for activity.

A study has been carried out to define the importance of the peripheral benzodiazepine receptor (PBR) as a binding site for a series of chlorin-type photosensitizers, pyropheophorbide-a ethers, the subject of a previous quantitative structure-activity relationship study by us. The effects of the PBR ligand PK11195 on the photodynamic activity have been determined in vivo for certain members of this series of alkyl-substituted ethers: two of the most active derivatives (hexyl and heptyl), the least active derivative (dodecyl [C12]) and one of intermediate activity (octyl [C8]). The photodynamic therapy (PDT) effect was inhibited by PK11195 for both of the most active derivatives, but no effect on PDT activity was found for the less active C12 or C8 ethers.

Parabolic quantitative structure-activity relationships and photodynamic therapy: application of a three-compartment model with clearance to the in vivo quantitative structure-activity relationships of a congeneric series of pyropheophorbide derivatives used as photosensitizers for photodynamic therapy.

An open three-compartment pharmacokinetic model was applied to the in vivo quantitative structure-activity relationship (QSAR) data of a homologous series of pyropheophorbide photosensitizers for photodynamic therapy (PDT). The physical model was a lipid compartment sandwiched between two identical aqueous compartments. The first compartment was assumed to clear irreversibly at a rate K0.

Design and construction of a light-delivery system for photodynamic therapy.

We have developed a device to divide the output from a dye laser into as many as eight beams of equal power with negligible total power loss. In this system, 630-nm s-plane polarized laser light was split by a series of highly polarization-sensitive plate beamsplitters. Each of the beams was coupled to a 200, 400, or 600 microm diameter optical fiber.

An in vivo quantitative structure-activity relationship for a congeneric series of pyropheophorbide derivatives as photosensitizers for photodynamic therapy.

An in vivo quantitative structure-activity relationship (QSAR) study was carried out on a congeneric series of pyropheophorbide photosensitizers to identify structural features critical for their antitumor activity in photodynamic therapy (PDT). The structural elements evaluated in this study include the length and shape (alkyl, alkenyl, cyclic, and secondary analogs) of the ether side chain. C3H mice, harboring the radiation-induced fibrosarcoma tumor model, were used to study three biological response endpoints: tumor growth delay, tumor cell lethality, and vascular perfusion.

Interstitial photoradiation therapy for primary solid tumors in pet cats and dogs.

Photoradiation therapy, a new method for treatment of solid malignant tumors, depends upon the tumor localization and retention of hematoporphyrin derivative, which is activated in vivo by light in the red region of the spectrum. As currently applied to cutaneous and s.c.

Photoradiation in the treatment of recurrent breast carcinoma.

Photoradiation, with the use of hematoporphyrin derivative (Hpd) activated by visible light in the red region of the spectrum, was an effective treatment for controlling local and regional chest wall recurrences of breast carcinoma. With sufficient time between iv injection of the drug and local activation with red light, cutaneous and subcutaneous masses were treated effectively without undue damage to overlying and adjacent skin. This high therapeutic ratio resulted from the ability to Hpd to accumulate and/or to be retained to a higher degree in malignant tissue than in many normal tissues.

Photoradiation therapy for the treatment of malignant tumors.

Administration of hematoporphyrin derivative i.v. followed by local exposure to red light has resulted in complete or partial response in 111 of 113 cutaneous or s.

Energetics and efficiency of photoinactivation of murine tumor cells containing hematoporphyrin.

Hematoporphryrin derivative at an intracellular concentration in TA-3 mouse mammary carcinoma cells of 0.6 or 0.9 mM required input of 3.

Identification of singlet oxygen as the cytotoxic agent in photoinactivation of a murine tumor.

Singlet oxygen, a metastable state of normal triplet oxygen, has been identified as the cytotoxic agent that is probably responsible for in vitro inactivation of TA-3 mouse mammary carcinoma cells following incorporation of hematoporphyrin and exposure to red light. This photodynamic inactivation can be completely inhibited by intracellular 1,3-diphenylisobenzofuran. This very efficient singlet oxygen trap is not toxic to the cells nor does it absorb the light responsible for hematoporphyrin activation.

Photoradiation therapy. II. Cure of animal tumors with hematoporphyrin and light.

Exposure of mouse and rat tumors of various types to more than 600 nm light 24 or 48 hours after an injection of hematoporphyrin resulted in a substantial number of long-term cures. Since hematoporphyrin is preferentially retained in tumor tissue, selective tumor destruction could be obtained. Light penetration studies and the high efficiency of this technique indicated its applicability even to certain deep-seated human tumors.