Application of Macro-Instrumented Indentation Test for Superficial Residual Stress and Mechanical Properties Measurement for HY Steel Welded T-Joints.

HY-80 and HY-100 steels, widely used in constructing large ocean vessels and submarine hulls, contain mixed microstructures of tempered bainite and martensite and provide high tensile strength and toughness. Weld integrity in HY steels has been studied to verify and optimize welding conditions. In this study, the T-joint weld coupons, HY80 and HY100, were fabricated from HY-80 and HY-100 steel plates with a thickness of 30 mm as base metals by submerged-arc welding.

CHD7 mutational analysis and clinical considerations for auditory rehabilitation in deaf patients with CHARGE syndrome.

Background: Otologic manifestations are one of the most consistent findings of CHARGE syndrome found in more than 90%. Since genetic analysis of the CHD7 gene has rarely been performed in previous reports dealing with ear abnormalities, the genotypic spectrum of CHD7 mutations was analyzed in deaf patients with CHARGE syndrome, and the clinical considerations concerning auditory rehabilitation were investigated.

Methods: Nine Korean patients with CHARGE syndrome showing profound hearing loss and semicircular canal aplasia were included.

Pou3f4 deficiency causes defects in otic fibrocytes and stria vascularis by different mechanisms.

DFN3, the most prevalent X-linked hearing loss, is caused by mutations in the POU3F4 gene. Previous studies in Pou3f4 knockout mice suggest that defective otic fibrocytes in the spiral ligament of the cochlear lateral wall may underlie the hearing loss in DFN3. To better understand the pathological mechanisms of the DFN3 hearing loss, we analyzed inner ears of Pou3f4-deficient mice during development.

A novel frameshift mutation of POU4F3 gene associated with autosomal dominant non-syndromic hearing loss.

Autosomal dominant mutations in the transcription factor POU4F3 gene are associated with non-syndromic hearing loss in humans; however, there have been few reports of mutations in this gene worldwide. We performed a mutation analysis of the POU4F3 gene in 42 unrelated Koreans with autosomal dominant non-syndromic hearing loss, identifying a novel 14-bp deletion mutation in exon 2 (c.662del14) in one patient.

Clinical and molecular characterizations of novel POU3F4 mutations reveal that DFN3 is due to null function of POU3F4 protein.

X-linked deafness type 3 (DFN3), the most prevalent X-linked form of hereditary deafness, is caused by mutations in the POU3F4 locus, which encodes a member of the POU family of transcription factors. Despite numerous reports on clinical evaluations and genetic analyses describing novel POU3F4 mutations, little is known about how such mutations affect normal functions of the POU3F4 protein and cause inner ear malformations and deafness. Here we describe three novel mutations of the POU3F4 gene and their clinical characterizations in three Korean families carrying deafness segregating at the DFN3 locus.