Use of cerrado plants as an alternative in the control of bacterial contamination in the alcoholic fermentation process.

Bacterial contamination causes irreparable losses in the performance of alcoholic fermentation. Antibiotics are used to control these microorganisms, but they generate residues and cause microbial resistance. Today the only commercial product used by the mills is hops, but it is very expensive. As an alternative, the objective of this work was to evaluate the feasibility of using extracts from plants grown in the Cerrado for antimicrobial control during an alcoholic fermentation to replace antibiotics. Hydraethanolic extracts of leaves and essential oil of the following species were tested: Schinus terebinthifolius Raddi, Serjania erecta, Serjania marginata, Campomanesia adamantium and Syzygium cumini. Only the extract of Serjania marginata did not show any activity against the bacterium Bacillus sp. Both the essential oils as well as the hydroalcoholic extracts of S. terebinthifolius and C. adamantium and the extract of S. erecta showed antibacterial activity without harming the yeast, with potential to replace the hops.

p.L18P: a novel IDUA mutation that causes a distinct attenuated phenotype in mucopolysaccharidosis type I patients.

Mucopolysaccharidosis type I is a rare autosomal recessive disorder caused by deficiency of α-l-iduronidase (IDUA) which leads to a wide spectrum of clinical severity. Here, we describe the case of four male patients who present the previously undescribed p.L18P mutation. Patient 1 (p.L18P/p.L18P) presents, despite multiple joint contractures, an attenuated phenotype. Patient 2 (p.L18P/p.W402X) was diagnosed at 4 years of age with bone dysplasia, coarse facies, limited mobility, claw hands and underwent bilateral carpal tunnel surgery at 6 years of age. Patients 3 and 4 (both p.L18P/p.L18P) are brothers. Patient 3 was diagnosed at 4 years of age, when presented claw hands, lower limb and shoulder pain, restricted articular movement and bilateral carpal tunnel syndrome. Patient 4 was diagnosed at 17 months of age when presented lower limb pain at night, respiratory allergy and repeated upper airways infections. Bioinformatics analysis indicates that p.L18P mutation reduces the signal peptide to 25 amino acids and alters its secondary structure. In conclusion, we report a new IDUA variant that alters the structure of the signal peptide, which likely impairs transport to lysosomes. Moreover, it leads to a distinct attenuated phenotype with mainly bone and cartilage symptoms, without visceromegalies, heart disease, or cognitive impairment.