An experimental study of dynamic characteristics of labyrinth seal

The fluid force due to labyrinth seal sometimes makes the turbomachineries unstable under higher rotating speed, higher pressure and higher power. Therefore, it is important to predict the magnitude and the direction of the fluid force and to evaluate the stability of the rotor system in design process. This paper shows the experimental results of the fluid force induced by a straight labyrinth seal and the rotordynamic coefficients calculated from the fluid force. Influences of the number of fins under the rotating speed, whirling speed, inlet pressure, and inlet tangential velocity are mainly investigated on a stability of the rotor system. The results show that increase of the number of fins makes the fluid force small and the rotor system stable, an increase of inlet pressure makes the fluid forces large and an increase of inlet tangential velocity makes the rotor system unstable

Abnormalities of the First Three Steps of Gait Initiation in Patients with Parkinson's Disease with Freezing of Gait

The purpose of this study was to investigate abnormalities of the first three steps of gait initiation in patients with Parkinson's disease (PD) with freezing of gait (FOG). Ten PD patients with FOG and 10 age-matched healthy controls performed self-generated gait initiation. The center of pressure (COP), heel contact positions, and spatiotemporal parameters were estimated from the vertical pressures on the surface of the force platform. The initial swing side of gait initiation was consistent among the trials in healthy controls but not among the trials in PD patients. The COP and the heel contact position deviated to the initial swing side during the first step, and the COP passed medial to each heel contact position during the first two steps in PD patients. Medial deviation of the COP from the first heel contact position had significant correlation with FOG questionnaire item 5. These findings indicate that weight shifting between the legs is abnormal and that medial deviation of the COP from the first heel contact position sensitively reflects the severity of FOG during the first three steps of gait initiation in PD patients with FOG

A novel COL4A1 variant associated with recurrent epistaxis and glioblastoma

COL4A1-related disorders are characterized by a higher incidence of cerebral hemorrhage than other hereditary cerebral small vessel diseases. Accumulating data have shown broad phenotypic variations, and extracerebral hemorrhages have been linked to these disorders. Moreover, the coexistence of neural tumors has been described. Here, we report a Japanese family with a novel COL4A1 variant, including a patient with recurrent epistaxis and glioblastoma

血清TARC/CCL17値は薬剤性過敏症症候群(DIHS) の早期診断および病勢の指標となりうる。

BACKGROUND:Drug-induced hypersensitivity syndrome (DIHS)/drug rash with eosinophilia and systemic symptoms (DRESS) is a serious acute drug reaction with fever, cutaneous eruption, lymphadenopathy, and several visceral dysfunctions. Eosinophilia is a common hematological abnormality in DIHS/DRESS suggesting that the Th2-type immune response is involved. Thymus and activation-regulated chemokine (TARC/CCL17) is a family of CC chemokines known to play an important role in Th2-mediated immune-inflammatory processes. OBJECTIVE:We investigated the pathogenic role of TARC in patients with DIHS. METHODS:Sera were obtained from 8 patients with DIHS, 7 patients with Stevens-Johnson syndrome/Toxic epidermal necrolysis (SJS/TEN), and 14 patients with drug-induced maculopapular exanthema (MPE). Serum TARC levels were measured by ELISA. TARC levels were then compared with clinical symptoms and various hematological parameters. In addition, a biopsy was taken from the lesional skin of patients with DIHS and stained with anti-TARC Ab and anti-CD11c Ab. RESULTS:Serum TARC levels in patients with DIHS were significantly higher than those in patients with SJS/TEN and MPE during the acute phase. Serum TARC levels in DIHS patients correlated with skin eruptions, serum sIL-2R levels, eosinophil counts, and serum IL-5 levels. Immunohistochemical staining revealed that TARC was mainly expressed on CD11c+ dermal dendritic cells in patients with DIHS. CONCLUSION:Serum TARC levels may be associated with the initial presentation of DIHS as well as disease activity during the course. Thus, they could be useful as an indicator for early diagnosis and assessment of disease activity in DIHS. CD11c+ dendritic cells may be the main source of TARC in patients with DIHS.博士（医学）・甲第597号・平成25年3月15日Copyright © 2012 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved

Nutrient-induced FNIP degradation by SCFβ-TRCP regulates FLCN complex localization and promotes renal cancer progression.

Folliculin-interacting protein 1 and 2 (FNIP1 and FNIP2) play critical roles in preventing renal malignancy through their association with the tumor suppressor FLCN. Mutations in FLCN are associated with Birt-Hogg-Dubé (BHD) syndrome, a rare disorder with increased risk of renal cancer. Recent studies indicated that FNIP1/FNIP2 double knockout mice display enlarged polycystic kidneys and renal carcinoma, which phenocopies FLCN knockout mice, suggesting that these two proteins function together to suppress renal cancer. However, the molecular mechanism functionally linking FNIP1/FNIP2 and FLCN remains largely elusive. Here, we demonstrated that FNIP2 protein is unstable and subjected to proteasome-dependent degradation via β-TRCP and Casein Kinase 1 (CK1)-directed ubiquitination in a nutrition-dependent manner. Degradation of FNIP2 leads to lysosomal dissociation of FLCN and subsequent lysosomal association of mTOR, which in turn promotes the proliferation of renal cancer cells. These results indicate that SCFβ-TRCP negatively regulates the FLCN complex by promoting FNIP degradation and provide molecular insight into the pathogenesis of BHD-associated renal cancer.福岡歯科大学2016年

ヒドウジョウミャク オ オンゾン シタ ヒオンゾン ビソクスイ セツジョジュツ ショウカカン ニュウセン シュジュツゴ ノ ヒゾウ ノ タイセキ ヘンカ

Aim: There is a paucity of information about changes in splenic volume after surgery. The aim of this study was to investigate postoperative changes in splenic volume（SV）and the factors influencing SV after spleen-preserving distal pancreatectomy（SPDP）with conservation of the splenic artery and vein （CSAV）, and after surgery of the digestive tract and breast.Methods: We investigated 113 patients who underwent SPDP with CSAV（n=7）, breast surgery （n=24）, colorectal surgery（n=45）, distal gastrectomy（n=27）and total gastrectomy（n=10）. SV changes were determined for three years after surgery using volumetry based on computed tomographic imaging, and splenic vein diameter changes after SPDP with CSAV were also determined.Results: Splenic vein diameter after SPDP with CSAV did not change during 3 years. SV did not change significantly during 3 years after SPDP with CSAV and distal gastrectomy. After breast and colorectal surgery, and total gastrectomy, SV was decreased.Conclusions: Postoperative SV changes differed according to the type of surgery. SV did not change significantly during 3 years after SPDP with CSAV

地域中核病院から病理解剖を依頼したALS

An 82-year-old man, who developed dysphagia several months ago, presented Tokushima University Hospital and was diagnosed of ALS in December 2019. The patient got gradually worse and became bedridden in May 2020. He was admitted into Tokushima University Hospital suffering an aspiration pneumonia in June 2020. The pneumonia rapidly improved with a treatment ; however, the patient failed to be treated at home against his wish and was transferred to Kaminaka Hospital. We accepted his wish for refusing mechanical ventilation or tube feeding. Later, we requested autopsy consent from the patient. He did not refuse our proposal ; therefore, we presearched transporters capable to deceased bodies and contacted the division of pathology in Tokushima University. 60 days later, the patient died due to a suddenly developed putamen hemorrhage. After getting the family's consent, as previously arranged, we transferred the deceased body to Tokushima University and accomplished an autopsy. Although the number of autopsies is declining, we suggest that hospital collaboration may help perform autopsies

TRPV4-dependent induction of a novel mammalian cold-inducible protein SRSF5 as well as CIRP and RBM3

Cold-inducible RNA-binding protein (CIRP) and RNA-binding motif protein 3 (RBM3) are two evolutionarily conserved RNA-binding proteins that are structurally related to hnRNPs and upregulated in response to moderately low temperatures in mammalian cells. Although contributions of splicing efficiency, the gene promoters activated upon mild hypothermia and the transcription factor Sp1 to induction of CIRP have been reported, precise mechanisms by which hypothermia and other stresses induce the expression of mammalian cold-inducible proteins (CIPs) are poorly understood. By screening the serine/arginine-rich splicing factors (SRSFs), we report that the transcript and protein levels of SRSF5 were increased in mammalian cells cultured at 32 °C. Expression of SRSF5 as well as CIRP and RBM3 were also induced by DNA damage, hypoxia, cycloheximide and hypotonicity. Immunohistochemical studies demonstrated that SRSF5 was constitutively expressed in male germ cells and the level was decreased in human testicular germ cell tumors. SRSF5 facilitated production of p19 H-RAS, and increased sensitivity to doxorubicin in human U-2 OS cells. Induction of CIPs was dependent on transient receptor potential vanilloid 4 (TRPV4) channel protein, but seemed independent of its ion channel activity. These findings indicate a previously unappreciated role for the TRP protein in linking environmental stress to splicing