Multi-scale characterization of lead-rich deposited particles originating from the fire of Notre-Dame de Paris.

Fire is a major hazard for built heritage. The fire at Notre-Dame on April 15, 2019 completely destroyed the woodframe and the lead roof (about 285 tons) almost entirely melted due to high temperatures. A part of the molten lead escaped into the atmosphere in the form of aerosols while the majority remains within cathedral enclosure in the form of deposits, metallic remains, spatters etc.

Does Atmospheric Corrosion Alter the Sound Quality of the Bronze Used for Manufacturing Bells?

Bells are made of bronze, an alloy of copper and tin. Art objects and musical instruments belong to tangible and intangible heritage. The effect of atmospheric alteration on their sound is not well documented.

Textile ageing due to atmospheric gases and particles in indoor cultural heritage.

Textile fibre degradation can be due to many factors. The most common cause is light exposure, but upon the lifespan of a textile, many other environmental factors are to be taken into account. This study focuses on the role of atmospheric compounds-both particulate and gaseous species-on natural textiles ageing, more specifically cotton, silk and wool.

Multi O- and S-isotopes as tracers of black crusts formation under volcanic and non-volcanic atmospheric conditions in Sicily (Italy).

The deterioration of monument or building stone materials is mostly due to the growth of black crusts that cause blackening and disaggregation of the exposed surface. This study reports on new oxygen (δO, δO and ΔO) and sulphur (δS, δS, δS, ΔS and ΔS) isotopic analyses of black crust sulphates formed on building stones in Sicily (Southern Italy). The measurements are used to identify the possible influence of volcanic emissions on black crust formation.

Reactivity of secondary phases in weathered limestone using isotopic tracers (D and O).

For a long time, limestone has been massively used in stone building and monuments because of its easy extraction and common presence in the landscape. On ancient monuments, mostly built in urban areas, it is exposed to urban-borne pollutants responsible for specific alteration mechanisms and weathering kinetics. Especially, the dissolution of calcite and the precipitation of new phases will affect the limestone pore network, modify the stones capillary properties, and influence the further alteration.

Assessment of indoor air quality for a better preventive conservation of some French museums and monuments.

Indoor air quality in museums and historical buildings is of great concern for curators, since it can be a source of various alterations on artworks. In spite of their importance, very few studies study simultaneously the concentration of main gaseous pollutants, the composition of suspended (PM), and deposited (DPM) particulate matter. The aim of this article is to carry out a first environmental assessment in French museums or monuments.

Water content of limestones submitted to realistic wet deposition: a CIME2 chamber simulation.

An experimental chamber (CIME2) has been specially designed to simulate wet atmospheric deposition on limestones used in Paris cultural heritage. This instrument is a complementary tool to CIME, a previously developed chamber dedicated to the simulation of dry atmospheric deposition on monuments and artifacts. The aim of this paper is to describe CIME2 and characterize the wet deposits produced inside it.

Characterisation of CIME, an experimental chamber for simulating interactions between materials of the cultural heritage and the environment.

An approach consisting in combining in situ and laboratory experiments is often favoured for investigating the mechanisms involved in the weathering of the materials of the cultural heritage. However, the realistic simulation in the laboratory of the environmental conditions ruling the interactions of atmospheric compounds with materials is a very complex task. The aim of this work is to characterise CIME, a new chamber specially built to simulate the interactions between materials of the cultural heritage and the environment.

Physico-chemical characterisation of glass soiling in rural, urban and industrial environments.

Glass materials are broadly used in the built environment (windows, facades, roofs, museum showcases, and solar panels) due to their optical (transparency) and thermal properties. Their interaction with the multiphase atmospheric medium results in a more or less pronounced transparency loss called soiling. This phenomenon leads to a loss of amenity of artefacts; consequently, high cleaning costs have to be supported by public and private entities.

Mapping the genetic and clinical characteristics of Gaucher disease in the Iberian Peninsula.

Background: Gaucher disease (GD) is due to deficiency of the glucocerebrosidase enzyme. It is panethnic, but its presentation reveals ethnicity-specific characteristics.

Methods: We evaluated the distribution, and clinical and genetic characteristics of GD patients in the Iberian Peninsula (IP).

Molecular analysis of Sanfilippo syndrome type C in Spain: seven novel HGSNAT mutations and characterization of the mutant alleles.

The Sanfilippo syndrome type C [mucopolysaccharidosis IIIC (MPS IIIC)] is caused by mutations in the HGSNAT gene, encoding an enzyme involved in heparan sulphate degradation. We report the first molecular study on several Spanish Sanfilippo syndrome type C patients. Seven Spanish patients, one Argentinean and three Moroccan patients were analysed.

Early stage of weathering of medieval-like potash-lime model glass: evaluation of key factors.

Purpose: Throughout history, a consequent part of the medieval stained glass windows have been lost, mostly because of deliberate or accidental mechanic destruction during war or revolution, but, in some cases, did not withstand the test of time simply because of their low durability. Indeed, the glasses that remain nowadays are for many in a poor state of conservation and are heavily deteriorated. Under general exposure conditions, stained glass windows undergo different kinds of weathering processes that modify their optical properties, chemistry, and structure: congruent dissolution, leaching, and particle deposition (the combination of those two leading together to the formation of neocrystallisations and eventually crusts).

Wet and dry atmospheric deposition on TiO(2) coated glass.

To prevent the soiling of glass window used in the built environment, the use TiO(2) coated products appears an important application matter. To test the cleaning efficiency and the sustainability of self-cleaning glass, a field experiment was conducted under real life condition, on a site representative of the background urban pollution. Samples of float glass, used as reference, and commercialized TiO(2) coated glasses were exposed to dry and wet atmospheric deposition during two years.

Dose-response function for the soiling of silica-soda-lime glass due to dry deposition.

Several exposure campaigns of silica-soda-lime window glass have been performed in 30 European sites and 1 in Canada in order to understand, quantify and model the phenomenon of soiling. In this purpose samples were exposed sheltered from the rain. Parallel to exposure, several meteorological parameters and pollution concentrations have been monitored.

Identification and characterization of SMPD1 mutations causing Niemann-Pick types A and B in Spanish patients.

Niemann-Pick disease (NPD) types A/B are both caused by a deficiency of lysosomal acid sphingomyelinase and display autosomal recessive inheritance. These two types of the disease were described according to the presence (type A) or absence (type B) of neurological symptoms. We present a molecular analysis of 19 Spanish NPD A/B patients and two from Maghreb.

Promising results of the chaperone effect caused by imino sugars and aminocyclitol derivatives on mutant glucocerebrosidases causing Gaucher disease.

Gaucher disease is an autosomal recessive disorder. It is characterized by the accumulation of glucosylceramide in lysosomes of mononuclear phagocyte system, attributable to acid beta-glucosidase deficiency. The main consequences of this disease are hepatosplenomegaly, skeletal lesions and, sometimes, neurological manifestations.

Haplotype analysis suggests a single Balkan origin for the Gaucher disease [D409H;H255Q] double mutant allele.

Gaucher disease is an autosomal recessive lysosomal storage disease that is mainly due to mutations in the GBA gene. Most of the mutant alleles described so far bear a single mutation. However, there are a few alleles bearing two or more DNA changes.

Maroteaux-Lamy syndrome: functional characterization of pathogenic mutations and polymorphisms in the arylsulfatase B gene.

Mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy syndrome) is an autosomal recessive lysosomal disorder caused by deficiency of N-acetylgalactosamine-4-sulfatase (ARSB), which is required for the degradation of dermatan sulfate. We recently reported mutational screening of 12 Spanish and 4 Argentinian MPS VI patients. In the present study, seven missense mutations (c.

An evolutionary and structure-based docking model for glucocerebrosidase-saposin C and glucocerebrosidase-substrate interactions - relevance for Gaucher disease.

Gaucher disease, the most prevalent lysosomal storage disorder, is principally caused by malfunction of the lysosomal enzyme glucocerebrosidase (GBA), a 497-amino acid membrane glycoprotein that catalyzes the hydrolysis of glucosylceramide to ceramide and glucose in the presence of an essential 84-residue activator peptide named saposin C (SapC). Knowledge of the GBA structure, a typical (beta/alpha)(8) TIM barrel, explains the effect of few mutations, directly affecting or located near the catalytic site. To identify new regions crucial for proper GBA functionality, we analyzed the interactions of the enzyme with a second (substrate) and a third (cofactor) partner.

Expression and characterization of 14 GLB1 mutant alleles found in GM1-gangliosidosis and Morquio B patients.

GM1-gangliosidosis and Morquio B disease are lysosomal storage disorders caused by beta-galactosidase deficiency attributable to mutations in the GLB1 gene. On reaching the endosomal-lysosomal compartment, the beta-galactosidase protein associates with the protective protein/cathepsin A (PPCA) and neuraminidase proteins to form the lysosomal multienzyme complex (LMC). The correct interaction of these proteins in the complex is essential for their activity.

Identification of a novel pseudodeficiency allele in the GLB1 gene in a carrier of GM1 gangliosidosis.

The term 'pseudodeficiency' is used in lysosomal storage diseases to denote the situation in which individuals show greatly reduced enzyme activity but remain clinically healthy. Pseudodeficiencies have been reported for several lysosomal hydrolases. GM1 gangliosidosis is a rare autosomal recessive lysosomal storage disorder caused by beta-galactosidase hydrolase deficiency as a result of mutations in the GLB1 gene.

Identification of the molecular defects in Spanish and Argentinian mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) patients, including 9 novel mutations.

Maroteaux-Lamy syndrome, or mucopolysaccharidosis VI (MPS VI), is an autosomal recessive lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-4-sulfatase or arylsulfatase B (ARSB). We aimed to analyze the spectrum of mutations responsible for the disorder in Spanish and Argentinian patients, not previously studied. We identified all the ARSB mutant alleles, nine of them novel, in 12 Spanish and 4 Argentinian patients.

Glycogen storage disease type II in Spanish patients: high frequency of c.1076-1G>C mutation.

Glycogen storage disease type II is an autosomal recessive disorder of glycogen metabolism due to deficiency of lysosomal acid alpha-glucosidase. We present the molecular and enzymatic analyses of 22 Spanish GSD II patients. Molecular analyses revealed nine novel mutations.