A5053419815- Ubiquitin and proteasome pathways
- Pituitary Gland Disorders and Treatments
- Pancreatic and Hepatic Oncology Research
- Gallbladder and Bile Duct Disorders
- Adrenal and Paraganglionic Tumors
- Autophagy in Disease and Therapy
- Cellular transport and secretion
- Lysosomal Storage Disorders Research
- Complement system in diseases
- Trypanosoma species research and implications
- Renal and related cancers
- Endoplasmic Reticulum Stress and Disease
- Muscle Physiology and Disorders
- Pancreatitis Pathology and Treatment
- Bone fractures and treatments
- Intestinal Malrotation and Obstruction Disorders
- Abdominal vascular conditions and treatments
- Urological Disorders and Treatments
- Gastrointestinal Tumor Research and Treatment
- Nuclear Structure and Function
- Mitochondrial Function and Pathology
- Neuroscience of respiration and sleep
- Pediatric Hepatobiliary Diseases and Treatments
- Insect symbiosis and bacterial influences
- Genomics and Chromatin Dynamics
Institute of Science Tokyo
2024-2025
Institute of Cell Biology
2025
Shanghai Institute for Science of Science
2024
Kyoto Prefectural University
2023-2024
Rikkyo University
2019-2024
Chukyo University
2024
Saitama Medical University
2024
Social Insurance Saitama Chuo Hospital
2024
Tokyo Institute of Technology
2014-2023
Hong Kong University of Science and Technology
2022
We have recently reported somatic mutations in the ubiquitin-specific protease USP8 gene a small series of adenomas patients with Cushing's disease.To determine prevalence and genotype-phenotype correlation large diagnosed disease.We performed retrospective, multicentric, genetic analysis 134 functioning 11 silent corticotroph using Sanger sequencing. Biochemical clinical features were collected examined within context mutational status USP8, new characterized by functional studies.A total...
Context Somatic mutations in the ubiquitin-specific peptidase USP8 gene were recently detected one- to two-third(s) of corticotroph adenomas Cushing's disease (CD). These may lead deubiquitination EGFR, thereby increasing EGFR signaling, which has been implicated ACTH hypersecretion. Objective Our objective was determine impact on clinicopathological features CD. Subjects and methods as well characteristics examined 60 including 15 Crooke's cell (CCAs), a rare histological variant presenting...
Nitric oxide (NO) is implicated in neuronal cell survival. However, excessive NO production mediates death, part via mitochondrial dysfunction. Here, we report that the ubiquitin ligase, MITOL, protects cells from damage caused by accumulation of S-nitrosylated microtubule-associated protein 1B-light chain 1 (LC1). S-nitrosylation LC1 induces a conformational change serves both to activate and promote its ubiquination indicating microtubule stabilization regulated through interaction with...
Differentiated muscle cells contain myofibrils and well-organized organelles, enabling powerful contractions. Muscle cell reorganization occurs in response to various physiological stimuli; however, the mechanisms behind this remodeling remain enigmatic due lack of a genetically trackable system. Previously, we reported that subset larval is remodeled into adult abdominal through an autophagy-dependent mechanism Drosophila . To unveil underlying remodeling, performed comparative time-course...
Differentiated muscle cells contain myofibrils and well-organized organelles, enabling powerful contractions. Muscle cell reorganization occurs in response to various physiological stimuli; however, the mechanisms behind this remodeling remain enigmatic due lack of a genetically trackable system. Previously, we reported that subset larval is remodeled into adult abdominal through an autophagy-dependent mechanism Drosophila . To unveil underlying remodeling, performed comparative time-course...
T-tubules are specialized invaginations of the plasma membrane essential for muscle contraction. While their physiological importance is well established, mechanisms underlying T-tubule formation remain elusive. Here, we identify LUBEL/RNF31, a ubiquitin E3 ligase responsible linear (M1-linked) ubiquitination, as key regulator biogenesis through proximity proteomics and RNAi screening in Drosophila. Loss LUBEL leads to Amphiphysin (Amph)-positive sheets instead tubular networks cells....
Abstract Ubiquitin-specific protease (USP) 25, belonging to the USP family of deubiquitinases, harbors two tandem ubiquitin-interacting motifs (UIMs), a ~20-amino-acid α-helical stretch that binds ubiquitin. However, role UIMs in USP25 remains unclear. Here we show UIM region Lys48-, but not Lys63-, linked ubiquitin chains, where played critical and cooperative role. Purified exhibited higher isopeptidase activity than chains. Mutations disrupted ubiquitin-binding ability resulted reduced...
Abstract A transverse-tubule (T-tubule) is an invagination of the plasma membrane penetrating deep into muscle cells. An extensive network T-tubules crucial for rapid and synchronized signal transmission from cell surface to entire sarcoplasmic reticulum Ca2+ release, leading contraction. are also indispensable formation positioning other organelles. Their structure physiological roles relatively well established; however, mechanisms shaping require further elucidation. Centronuclear...
The nuclear lamina (NL) plays various roles and participates in integrity, chromatin organization, transcriptional regulation. Lamin proteins, the main components of NL, form a homogeneous meshwork structure under envelope. Lamins are essential, but it is unknown whether their distribution important for function. Here, we found that PIGB, an enzyme involved glycosylphosphatidylinositol (GPI) synthesis, responsible lamin Drosophila. Loss PIGB resulted heterogeneous distributions B-type...
Beckwith–Wiedemann syndrome (BWS) manifests distinctive features, such as macroglossia, overgrowth, and abdominal wall defects. In this report, we describe a case of BWS in an extremely low birth weight infant diagnosed at three months after because the intensive care for weight. A female was delivered 24 weeks 6 days gestation with 845 g. After birth, significant small intestinal intra-umbilical prolapse observed, closure using sutureless method performed on day zero. Careful neonatal...
The daggertooth Anotopterus pharao (Aulopiformes: Anotopteridae) is a large, piscivorous predator that lives within the epipelagic zone at night. In this species, distribution of retinal ganglion cells has been examined. An isodensity contour map shows concentrate in slightly ventral region temporal retina. high cell density contains 4.07 x 10(3) mm(-2), and resulting visual acuity 3.5 cycles deg(-1). Outside area centralis, conspicuously large (LGCs) are observed margin LGCs regularly...
Aberrant subvesical bile ducts are rare structural anomaly located in the peri-hepatic gallbladder fossa. This duct poses risk for intraoperative injury resulting clinically relevant leakage. was detected by preoperative magnetic resonance cholangiopancreatography a 52-year old woman with polypoid tumor harboring to be cancer. During open cholecystectomy full thickness dissection, aberrant identified fluorescent cholangiography (IFC), and dissected safely. mostly found unexpectedly as intra...
[08:00 – 08:10]We have recently reported that somatic mutations in the ubiquitin-specific peptidase USP8 are present corticotropinomas of patients with Cushing's disease and these reduced interaction 14 3-3. Mutant exhibited higher deubiquitination activity promoted POMC expression an EGFR-depending fashion (Reincke et al., Nat. Genetics 2014). To further study prevalence mutations, we analyzed 145 ACTH-producing diagnosed from 8 different centers by Sanger sequencing.