ABSTRACT
Prolyl 3-hydroxylation is a rare collagen type I post translational modification in fibrillar collagens. The primary 3Hyp substrate sites in type I collagen are targeted by an endoplasmic reticulum (ER) complex composed by cartilage associated protein (CRTAP), prolyl 3-hydroxylase 1 (P3H1) and prolyl cis/trans isomerase B, whose mutations cause recessive forms of osteogenesis imperfecta with impaired levels of α1(I)3Hyp986. The absence of collagen type I 3Hyp in wild type zebrafish provides the unique opportunity to clarify the role of the complex in vertebrate. Zebrafish knock outs for crtap and p3h1 were generated by CRISPR/Cas9. Mutant fish have the typical OI patients' reduced size, body disproportion and altered mineralization. Vertebral body fusions, deformities and fractures are accompanied to reduced size, thickness and bone volume. Intracellularly, collagen type I is overmodified, and partially retained causing enlarged ER cisternae. In the extracellular matrix the abnormal collagen type I assembles in disorganized fibers characterized by altered diameter. The data support the defective chaperone role of the 3-hydroxylation complex as the primary cause of the skeletal phenotype.
Subject(s)
Collagen Type II/metabolism , Collagen Type I/metabolism , Extracellular Matrix Proteins/genetics , Osteogenesis Imperfecta/genetics , Prolyl Hydroxylases/genetics , Animals , CRISPR-Cas Systems , Cyclophilins/genetics , Disease Models, Animal , Gene Knockout Techniques , Hydroxylation , Osteogenesis Imperfecta/metabolism , Phenotype , Prolyl Hydroxylases/chemistry , Zebrafish , Zebrafish Proteins/chemistry , Zebrafish Proteins/geneticsABSTRACT
The authors report a clinical investigation on 49 patients: 9 with acute leukemia and 40 with chronic leukemia. A file suggested by OMS was used to collect all data about patients and case histories. Patients suffering from acute leukemia, admitted to sterile rooms, were submitted to a simple clinical investigation because of their severe general conditions and to avoid any contamination. Except for a case of stomatitis caused by methotrexate, no oral disease was observed. Patients with LMC, examined with CPITN index, revealed a high presence of tartar (44.87%) and periodontal pockets, 4-5 mm deep, (23.72%). DMFT was 22.60, a rather high value caused by the large number of missing teeth. The data analysis evidences the severe clinical conditions of the patients and the urgent necessity for proper prophylactic and therapeutic treatment.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Leukemia, Myeloid/diagnosis , Oral Health , Acute Disease , Adult , Aged , Aged, 80 and over , DMF Index , Female , Health Services Needs and Demand , Humans , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/complications , Leukemia, Myeloid/complications , Male , Middle Aged , Periodontal Diseases/diagnosis , Periodontal Diseases/epidemiology , Periodontal IndexABSTRACT
The occurrence of opportunistic pathogens and the concentration of some antimicrobial factors in the oral cavity of both acute and chronic leukaemia patients were studied. Enterobacteria were isolated from both dental plaque and crevicular fluid of all the groups examined, with few differences between healthy volunteers and leukaemic subjects; yeasts were found in both the crevicular fluid and the dental plaque samples of chronic leukaemia patients, but only in the plaque of healthy volunteers. Acute leukaemia patients did not have yeasts, but they were the only group colonized by the pseudomonads. IgA and N-acetyl-D-glucosaminidase (NAGase) significantly increased in chronic leukaemia patients compared with controls, whilst lysozyme seemed to present no marked differences for all groups. A further increase in NAGase concentration and an elevation in lysozyme content of saliva was observed for chronic leukaemia patients with severe periodontal lesions.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/complications , Leukemia, Myeloid, Acute/complications , Mouth/microbiology , Opportunistic Infections/microbiology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , Acetylglucosaminidase/metabolism , Dental Plaque/microbiology , Enterobacteriaceae/isolation & purification , Gingival Crevicular Fluid/microbiology , Humans , Immunoglobulin A/metabolism , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/microbiology , Leukemia, Myeloid, Acute/microbiology , Muramidase/metabolism , Precursor Cell Lymphoblastic Leukemia-Lymphoma/microbiology , Pseudomonas/isolation & purification , Saliva/enzymology , Saliva/metabolism , Yeasts/isolation & purificationABSTRACT
Se refiere a las propiedades germicidas de metales pesados, especialmente la plata, usados con los dosajes adecuados para no afectar al organismo humano. Comienza con una reseña histórica sobre este tema para luego describir el método de electrocatadinización. Resalta la necesidad de calcular con precisión la dosis de plata utilizada y el tiempo que permanece en contacto con el agua. Expone las ventajas de este método y afirma que es inocuo para la salud
Subject(s)
Water Purification , Water Disinfectants , SilverABSTRACT
Se refiere a las propiedades germicidas de metales pesados, especialmente la plata, usados con los dosajes adecuados para no afectar al organismo humano. Comienza con una reseña histórica sobre este tema para luego describir el método de electrocatadinización. Resalta la necesidad de calcular con precisión la dosis de plata utilizada y el tiempo que permanece en contacto con el agua. Expone las ventajas de este método y afirma que es inocuo para la salud
Subject(s)
Water Disinfectants , Silver , Water PurificationABSTRACT
Dental and periodontal conditions of 80 handicapped adults were examined. The epidemiological survey was made by following the WHO suggestions and showed that there are patients in great need of primary and secondary prevention. The results of the research point out a DMFT of 18.78 and extractions were practically the only dental therapy received by these specific patients. Periodontal diseases were surveyed by the use of the CPITN index, and exhibited high values of dental calculus in younger. Furthermore, as the patients increased in age, periodontal pockets appeared and edentulous patients increased.
