Construction of self-cloning Aspergillus oryzae strains with high production of multiple biomass-degrading enzymes on solid-state culture.

Filamentous fungi produce numerous industrially important enzymes. Among them, Aspergillus oryzae-derived enzymes are widely used in various fermentation applications. In this study, we constructed self-cloning strains that overproduce multiple biomass-degrading enzymes under the control of a strong promoter of α-amylase-coding gene (amyB) using the industrial strain A.

Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan.

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation.

Telefitting of Nucleus Cochlear Implants: A Feasibility Study.

Purpose This study aims to describe the recommended equipment and procedures required for successful telefitting, based on our experience, document and evaluate patient satisfaction with telefitting, and assess its clinical usefulness and address the existing issues. Method Twenty (seven children and 13 adults) individuals who lived far from cochlear implant (CI) centers and who were Nucleus CI users underwent conventional face-to-face fitting and telefitting. We examined the participants' subjective satisfaction and cost and time saved with the telefitting experience.

Features predicting treatment failure in pediatric acute otitis media.

Objectives: To facilitate better antibiotic stewardship, we conducted this clinical trial to identify the prognostic features of treatment failure in pediatric acute otitis media (AOM).

Study: Design: This is a randomized, parallel-group, open-label, comparative clinical trial.

Subjects And Methods: Children with AOM and aged between 1 month and 5 years were enrolled.

The Prevalence and Clinical Characteristics of -Associated Autosomal Dominant Hearing Loss.

is well known as a causative gene for autosomal dominant mid-frequency hearing loss observed in various populations. In this study, we performed next-generation sequencing analysis of a large Japanese hearing loss cohort, including eight hundred and twelve (812) subjects from unrelated autosomal dominant hearing loss families, to estimate the prevalence and phenotype-genotype correlations in patients with mutations. The prevalence of mutations in Japanese autosomal dominant sensorineural hearing loss families was found to be 3.

Efficient production of recombinant tannase in Aspergillus oryzae using an improved glucoamylase gene promoter.

A tannase-encoding gene, AotanB, from Aspergillus oryzae RIB40 was overexpressed in A. oryzae AOK11 niaD-deficient mutant derived from an industrial strain under the control of an improved glucoamylase gene promoter PglaA142. The recombinant tannase, designated as rAoTanBO, was produced efficiently as an active extracellular enzyme.

Deterioration in Distortion Product Otoacoustic Emissions in Auditory Neuropathy Patients With Distinct Clinical and Genetic Backgrounds.

Objectives: Auditory neuropathy (AN) is a clinical disorder characterized by the absence of auditory brainstem response and presence of otoacoustic emissions. A gradual loss of otoacoustic emissions has been reported for some cases of AN. Such cases could be diagnosed as cochlear hearing loss and lead to misunderstanding of the pathology when patients first visit clinics after the loss of otoacoustic emissions.

Cellular responses to the expression of unstable secretory proteins in the filamentous fungus Aspergillus oryzae.

Filamentous fungi are often used as cell factories for recombinant protein production because of their ability to secrete large quantities of hydrolytic enzymes. However, even using strong transcriptional promoters, yields of nonfungal proteins are generally much lower than those of fungal proteins. Recent analyses revealed that expression of certain nonfungal secretory proteins induced the unfolded protein response (UPR), suggesting that they are recognized as proteins with folding defects in filamentous fungi.

Clinical Review of Cochlear Implantation Performed at Sapporo Medical University Hospital.

More than 20 years have passed since cochlear implantation (CI) was first introduced in Japan. We began CI at the Sapporo Medical University Hospital in 1988; since then, up to the first half of 2015, we have performed CI on 280 ears. In patients aged less than and those aged over 18 years, 121 and 159 ears, respectively, have undergone surgery.

Diagnosis and Treatment of Obstructive Sleep Apnea Syndrome in Children.

Sleep is important for children pertaining to their physical and mental growth. Obstructive sleep apnea syndrome (OSAS) in children has been shown to have different effects as compared to OSAS in adults, including deficits in cognition and neuropsychological functions, hyperactivity, ADHD, behavior problems, aggressive behavior, learning problems and nocturnal enuresis. Hypertrophy of the adenoids and tonsils is a major cause of OSAS in children; therefore, adenotonsillectomy may decrease the effects of OSAS pertaining to physical and mental growth.

Molecular Impairment Mechanisms of Novel OPA1 Mutations Predicted by Molecular Modeling in Patients With Autosomal Dominant Optic Atrophy and Auditory Neuropathy Spectrum Disorder.

Hypothesis: Different missense mutations of the optic atrophy 1 gene (OPA1) identified in optic atrophy patients with auditory neuropathy spectrum disorder (ANSD) induce functional impairment through different molecular mechanisms.

Background: OPA1 is the gene responsible for autosomal dominant optic atrophy (ADOA), but some of its mutations are also associated with ANSD. OPA1 is a member of the GTPase family of proteins and plays a key role in the maintenance of mitochondrial activities that are dependent on dimer formation of the protein.

Epithelial barrier and antigen uptake in lymphoepithelium of human adenoids.

Invasion of antigens through the mucosal surface can be prevented by the common mucosal immune system, including Peyer's patches (PPs) and nasopharyngeal-associated lymphoreticular tissue (NALT). The adenoids (nasopharyngeal tonsils) comprise one of the NALTs and constitute the major part of Waldeyer's lymphoid ring in humans. However, the role of the lymphoepithelium, including M cells and dendritic cells (DCs), in the adenoids is unknown compared with the epithelium of PPs.

Audiological chronological findings in children with congenital anomalies of the central nervous system.

Objective: To determine the frequency of hearing impairment in children with congenital anomalies of the central nervous system (CNS) by using detailed audiological evaluation methods.

Methods: The patients were 78 children with congenital anomalies of the CNS with a mean age of 29.5 months.

Non-invasive positive pressure ventilation for laryngeal contraction disorder during sleep in multiple system atrophy.

We examined the usefulness of non-invasive positive pressure ventilation (NPPV) in the management of nocturnal laryngeal stridor associated with vocal cord dysfunction in five cases of multiple system atrophy (MSA). First, the patients were investigated during sleep induced by a minimal dose of propofol. Laryngoscopy showed paradoxical vocal cord movement resulting in inspiratory stridor.