A5083664157- Autoimmune Bullous Skin Diseases
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Urticaria and Related Conditions
- Skin and Cellular Biology Research
- Cell Adhesion Molecules Research
- Dermatological and Skeletal Disorders
- Cellular Mechanics and Interactions
- Cancer and Skin Lesions
- Hair Growth and Disorders
- Nail Diseases and Treatments
- Wnt/β-catenin signaling in development and cancer
- Genetic and rare skin diseases.
- Platelet Disorders and Treatments
- Cutaneous lymphoproliferative disorders research
- Oral and gingival health research
- Autoimmune and Inflammatory Disorders
- Plant Reproductive Biology
- RNA regulation and disease
- Eosinophilic Disorders and Syndromes
- Vascular Tumors and Angiosarcomas
- Oral Health Pathology and Treatment
- melanin and skin pigmentation
- Silk-based biomaterials and applications
- CNS Lymphoma Diagnosis and Treatment
- Monoclonal and Polyclonal Antibodies Research
Hokkaido University
2015-2024
University Medical Center Freiburg
2010-2012
University of Freiburg
2011-2012
Kin-ikyo Chuo Hospital
2001-2006
Sapporo City General Hospital
2004
Type XVII collagen (COL17) is a transmembrane protein located at the epidermal basement membrane zone. COL17 deficiency results in premature hair aging phenotypes and junctional epidermolysis bullosa. Here, we show that plays central role regulating interfollicular epidermis (IFE) proliferation. Loss of leads to transient IFE hypertrophy neonatal mice owing aberrant Wnt signaling. The replenishment COL17-null reverses proliferative phenotype altered Physical abolishes membranous basal cells...
Bullous pemphigoid (BP), a common autoimmune blistering disease, is increasing in incidence and conveys high mortality. Detection of autoantibodies targeting the noncollagenous 16A (NC16A) domain type XVII collagen using enzyme-linked immunosorbent assay (ELISA) has demonstrated sensitivity specificity for diagnosing BP. We have developed rapid, low-cost, widely applicable ELISA-based system to detect NC16A antibody then diagnose monitor BP disease activity piece filter paper, wax-printer,...
Pemphigoid diseases refer to a group of severe autoimmune skin blistering characterized by subepidermal and loss dermal-epidermal adhesion induced autoantibody immune cell infiltrate at the junction upper dermis. Here, we explore role cell-secreted serine protease, granzyme B, in pemphigoid disease pathogenesis using three independent murine models. In all models, B knockout or topical pharmacological inhibition significantly reduces total area compared controls. vivo vitro studies show that...
Complement activation and subsequent recruitment of inflammatory cells at the dermal/epidermal junction are thought to be essential for blister formation in bullous pemphigoid (BP), an autoimmune blistering disease induced by autoantibodies against type XVII collagen (COL17); however, this theory does not fully explain pathological features BP. Recently, involvement complement-independent pathways has been proposed. To directly address question necessity complement formation, we generated...
Abstract The pemphigoid group is a category of autoimmune subepidermal blistering diseases in which autoantibodies deposit linearly at the epidermal basement membrane zone ( BMZ ). main subtypes mediated by immunoglobulin G are bullous BP ), mucous MMP ) and epidermolysis bullosa acquisita EBA To establish first guidelines approved Japanese Dermatological Association for management diseases, Committee Guidelines Management Pemphigoid Diseases (Including was founded as part Study Group Rare...
Bullous pemphigoid (BP) might be drug-induced. The present study evaluated the relationship between BP and dipeptidyl peptidase-4 inhibitors (DPP4Is).We recruited patients diagnosed with at Ogaki Municipal Hospital from 1 December 2009 through 31 2017. We retrospectively collected data medical records divided into two groups based on whether they received DPP4Is. Additionally, we determined incidence of in who were first prescribed DPP4Is our hospital during period.Of 168 BP, 133 (79.1%)...
Harlequin ichthyosis (HI), which is the most severe genodermatosis, caused by loss-of-function mutations in ABCA12, a member of ATP-binding cassette transporter family. To investigate pathomechanism HI and function ABCA12 protein, we generated ABCA12-deficient mice (Abca12 −/− ) targeting Abca12 . closely reproduce human phenotype, showing marked hyperkeratosis with eclabium skin fissure. Lamellar granule abnormalities defective ceramide distribution were remarkable epidermis. Skin...
Abstract In bullous pemphigoid (BP), the most prevalent autoimmune blistering disease, type XVII collagen (COL17) is targeted by circulating autoantibodies. BP thought to be an autoantibody-mediated complement-fixing and a juxtamembranous noncollagenous 16A (NC16A) domain spanning Glu490 Arg566 was proved main pathogenic region on COL17, although precise epitopes within NC16A have not been elucidated. this study, we showed that injection of rabbit IgG Abs targeting Asp522 Gln545 induced skin...
Bullous pemphigoid (BP), the most common autoimmune blistering disease, is caused by autoantibodies against type XVII collagen (COL17). To establish an active stable BP animal model that demonstrates persistent inflammatory skin lesions initiated anti-human COL17 Abs, we used COL17-humanized (COL17(m-/-,h+)) mice recently produced. First, generated immunodeficient Rag-2(-/-)/COL17-humanized crossing Rag-2(-/-) with mice. Then, splenocytes from wild-type had been immunized grafting of human...
Abstract As a type II transmembrane protein in basal keratinocytes, collagen XVII provides stable adhesion between epidermis and dermis the skin. Its ectodomain can be shed from cell surface, autoantibodies certain blistering diseases preferentially recognize form. Major epitopes of are clustered within juxtamembranous noncollagenous 16th A domain, shedding occurs this region, suggesting that cleavage generates neoepitopes. However, candidate sites have been controversial, mechanism...
Attempts to treat congenital protein deficiencies using bone marrow-derived cells have been reported. These efforts based on the concepts of stem cell plasticity. However, it is considered more difficult restore structural proteins than secretory enzymes. This study aims clarify whether marrow transplantation (BMT) treatment can rescue epidermolysis bullosa (EB) caused by defects in keratinocyte proteins. BMT adult collagen XVII (Col17) knockout mice induced donor-derived keratinocytes and...