The promyelocytic leukemia (PML) protein is the main component of PML nuclear bodies, which have many functions in a wide range of cell types. Until recently, PML was not known to have a function in the nervous system or even be expressed in the brain. However, recent reports have changed that view. PML is found in neurons and functions in many aspects of the nervous system, including brain development, circadian rhythms, plasticity, and the response to proteins that cause neurodegenerative disorders. While the investigation of PML in the brain is still in its infancy, it promises to be a fascinating subject that will contribute to our understanding of the brain. Here we summarize what is known about PML expression and function in the brain and highlight both discrepancies in the field and areas that are particularly important to future research.
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http://dx.doi.org/10.3389/fonc.2013.00242 | DOI Listing |
Cureus
October 2024
Division of Hematology and Cell Therapy, Department of Internal Medicine Ⅲ, Yamagata University, Yamagata, JPN.
A female patient aged in her 50s had presented with the onset of follicular lymphoma (FL) with left mandibular swelling, with a pathological grade of 1 and clinical stage of Ⅳ (Ann Arbor staging). Cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (R-CHOP) resulted in complete molecular remission (CMR). The patient experienced two recurrences, and treatments were successful; however, the side effect of continuous lymphocytopenia existed eight years after the onset.
View Article and Find Full Text PDFNeurology
December 2024
From the Department of Neurology (Y.W., S.W., M.L.), West China Hospital, Sichuan University, Chengdu, China; The George Institute for Global Health (Y.W., T.M., S.Y., C.C., L.L., Z.Z., C.D., J.P.C., C.S.A., X.C.), Faculty of Medicine, University of New South Wales, Sydney, Australia; Department of Cardiovascular Sciences and NIHR Leicester Biomedical Research Centre (T.G.R.), Leicester, United Kingdom; University of Sydney (R.I.L.); Department of Clinical Medicine (C.D.), Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, Australia; Division of Neuroimaging Sciences (G.M., J.M.W.), Centre for Clinical Brain Sciences and Centre in the UK Dementia Research Institute, University of Edinburgh, United Kingdom; Department of Neurology (J.P.C., C.S.A.), Royal Prince Alfred Hospital, Sydney Health Partners, Australia; Department of Preventive Medicine and Public Health (H.A.), Faculty of Medicine, Fukuoka University, Fukuoka, Japan; Neurology Department (Y.H.), Peking University First Hospital, Beijing, China; Neurology Department (J.S.K.), Asan Medical Centre, Seoul, South Korea; Neurology and Psychiatry Department (P.M.L.), Clinica Alemana de Santiago, Facultad de Medicina Universidad del Desarrollo, Chile; Neurology Department (T.-H.L.), Chang Gung Memorial Hospital, Taipei, Taiwan; Neurology Department (C.L., M.W.P.), John Hunter Hospital and Hunter Medical Research Institute, Newcastle, Australia; Brazilian Stroke Network (S.C.M.), Hospital de Clínicas de Porto Alegre, Hospital Moinhos de Vento, Brazil; Neurology Department (J.D.P.), Christian Medical College, Ludhiana, India; Department of Neuroscience and Behavioral Sciences (O.M.P.-N.), Ribeirao Preto School of Medicine, Brazil; School of Medicine (V.K.S.), National University of Singapore and Division of Neurology (V.K.S.), National University Hospital, Singapore; Department of Cerebrovascular Disease (T.H.N.), 115 Hospital, Ho Chi Minh City, Vietnam; Shanghai Institute for Hypertension (J.W.), Rui Jin Hospital and Shanghai Jiaotong University; and Institute of Science and Technology for Brain-inspired Intelligence (C.S.A.), Fudan University, Shanghai, China.
Pathol Int
December 2024
Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by JC virus (JCV) infection. Although recognized as an AIDS complication in the 1980s, PML has emerged as a serious adverse event of immunosuppressive therapies since 2005, particularly disease-modifying drugs (DMDs) for multiple sclerosis (MS). PML can also occur in patients with collagenous diseases receiving steroid therapy or with age-related immunosuppression.
View Article and Find Full Text PDFBMC Neurol
November 2024
Department of Microbiology and Infection Control, University Hospital of North Norway, Tromsø, Norway.
Background: Progressive multifocal leukoencephalopathy (PML) is a severe opportunistic brain disease caused by lytic JC polyomavirus (JCPyV) replication in oligodendrocytes. Although JCPyV infection is common in the general population, PML almost exclusively occurs in patients immunocompromised due to untreated HIV/AIDS, haematological malignancies, primary immunodeficiencies, solid organ transplantation, or immunomodulatory treatment of autoimmune diseases. There is no effective antiviral treatment, and recovery depends on immune reconstitution.
View Article and Find Full Text PDFCureus
September 2024
Stroke, Russells Hall Hospital, Dudley, GBR.
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal neurological disorder caused by the John Cunningham virus (JCV). It affects immunocompromised individuals, leading to brain demyelination. Diagnosis involves MRI scans and JCV detection in cerebrospinal (CSF) fluid.
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