Amir Dori

ORCID: 0000-0003-4633-1481
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About
Contact & Profiles
Research Areas
  • Muscle Physiology and Disorders
  • Cholinesterase and Neurodegenerative Diseases
  • Blood Coagulation and Thrombosis Mechanisms
  • Botulinum Toxin and Related Neurological Disorders
  • Amyloidosis: Diagnosis, Treatment, Outcomes
  • Peripheral Neuropathies and Disorders
  • Hereditary Neurological Disorders
  • Neurogenetic and Muscular Disorders Research
  • Inflammatory Myopathies and Dermatomyositis
  • Genetic Neurodegenerative Diseases
  • Extracellular vesicles in disease
  • Nicotinic Acetylcholine Receptors Study
  • Amyotrophic Lateral Sclerosis Research
  • Dermatological and Skeletal Disorders
  • Neuroscience and Neuropharmacology Research
  • Peripheral Nerve Disorders
  • Computational Drug Discovery Methods
  • Myasthenia Gravis and Thymoma
  • Pain Mechanisms and Treatments
  • Neurogenesis and neuroplasticity mechanisms
  • Pesticide Exposure and Toxicity
  • Cardiomyopathy and Myosin Studies
  • Eosinophilic Disorders and Syndromes
  • Muscle activation and electromyography studies
  • Myofascial pain diagnosis and treatment

Sheba Medical Center
2016-2025

Tel Aviv University
2016-2025

Rabin Medical Center
2024

Rafael Advanced Defense Systems (Israel)
2024

Academic College of Tel Aviv-Yafo
2024

Faculty (United Kingdom)
2024

Humanitas University
2023

Washington University in St. Louis
2013-2019

Alzheimer's Association of Israel
2009

Ben-Gurion University of the Negev
1999-2006

Mislocalization of the predominantly nuclear RNA/DNA binding protein, TDP-43, occurs in motor neurons ~95% amyotrophic lateral sclerosis (ALS) patients, but contribution axonal TDP-43 to this neurodegenerative disease is unclear. Here, we show accumulation intra-muscular nerves from ALS patients and axons human iPSC-derived patient, as well neuromuscular junctions (NMJs) a mislocalization mouse model. In axons, hyper-phosphorylated promotes G3BP1-positive ribonucleoprotein (RNP) condensate...

10.1038/s41467-021-27221-8 article EN cc-by Nature Communications 2021-11-25

Transthyretin amyloidosis (ATTR amyloidosis) is a rare, life-threatening disease caused by the accumulation of variant or wild-type (ATTRwt transthyretin amyloid fibrils in heart, peripheral nerves, and other tissues organs.Established 2007, Amyloidosis Outcomes Survey (THAOS) largest ongoing, global, longitudinal observational study patients with ATTR amyloidosis, including both inherited disease, asymptomatic carriers pathogenic TTR mutations. This descriptive analysis examines baseline...

10.1186/s13023-022-02359-w article EN cc-by Orphanet Journal of Rare Diseases 2022-06-18

To explore the scope and significance of alternate promoter usage its putative inter-relationship to alternative splicing, we searched expression sequence tags for 5′ region acetylcholinesterase (ACHE) genes. Three five novel first exons were identified in human mouse ACHE genes, respectively. Reverse transcription-PCR situ hybridization validated most predicted transcripts, analyses corresponding genomic DNA regions suggest evolutionarily conserved promoters each identified. Distinct tissue...

10.1074/jbc.m402752200 article EN cc-by Journal of Biological Chemistry 2004-07-01

Abstract Zinc maintains a diverse array of functions in the mammalian central nervous system as key component numerous enzymes, via its role activation transcription factors, and neuroregulator, modulating neuronal receptors such N‐methyl‐D‐aspartate γ‐aminobutyric acid. has dark side, however, with massive influx Zn 2+ to neurons considered be factor death secondary ischemia seizure. Several different putative zinc transporters, ZnT‐1–4, have recently been identified characterized. Among...

10.1002/cne.10224 article EN The Journal of Comparative Neurology 2002-04-25

Transthyretin amyloidosis (ATTR amyloidosis) is a progressive, multi-systemic disease with wild-type (ATTRwt) and hereditary (ATTRv) forms. Over 130 variants associated ATTRv have been identified, although little known about the majority of these genotypes. This analysis examined phenotypic characteristics symptomatic patients enrolled in Amyloidosis Outcomes Survey (THAOS) four less frequently reported pathogenic genotypes: F64L (c.250T>C, p.F84L), I68L (c.262A>T, p.I88L), I107V...

10.1371/journal.pone.0292435 article EN cc-by PLoS ONE 2024-01-19

Proliferation and differentiation of mammalian central nervous system progenitor cells involve concertedly controlled transcriptional alternative splicing modulations. Searching for the developmental implications this programming, we manipulated specific acetylcholinesterase (AChE) splice variants in embryonic mouse brain. In wild type mice, 'synaptic' AChE-S appeared migrating neurons, whereas C-terminus cleaved off stress-induced AChE-R variant associated with migratory radial glial...

10.1093/cercor/bhh145 article EN Cerebral Cortex 2004-08-06

Transient systemic inflammation has been shown to cause altered behavior both in humans and laboratory animals through activation of microglia heightened level cytokines detected the brain body. Furthermore, activated increased have associated with sudden clinical deterioration demented people or aged patients upon inflammation. Whilst it is increasingly becoming clear role transient promoting dementia individuals, still a matter debate whether prolonged might persistently modify brain. In...

10.3389/fnmol.2013.00046 article EN cc-by Frontiers in Molecular Neuroscience 2013-01-01

Abstract Duchenne muscular dystrophy (DMD) is a progressive, lethal, X-linked disease of skeletal and cardiac muscles caused by mutations in the dystrophin gene. Loss leads to muscle fiber damage impairment satellite cell asymmetric division, which are essential for regeneration. These processes ultimately result wasting replacement degenerating fibrogenic cells, process that generation fibrotic tissues. Preimplantation factor (PIF) an evolutionary conserved 15-amino acid peptide secreted...

10.1038/s41419-019-1307-9 article EN cc-by Cell Death and Disease 2019-01-28

Initial clinical trials and surveillance data have shown that the most commonly administered BNT162b2 COVID-19 mRNA vaccine is effective safe. However, several cases of vaccine-induced mild to moderate adverse events were recently reported. Here, we report a rare case myositis after injection first dose into left deltoid muscle 34-year-old, previously healthy woman who presented progressive proximal weakness, dysphagia, dyspnea with respiratory failure. One month vaccination, expression was...

10.3390/vaccines10071135 article EN cc-by Vaccines 2022-07-16

10.1080/13506129.2023.2229484 article EN cc-by-nc-nd Amyloid 2023-07-17

Changes in neuronal conduction are common disease states affecting peripheral nerves. These alterations can significantly impact nerve function and lead to sensorimotor disabilities. In vivo electromyography recording is a well-established electrophysiological method that has been used for decades assess sensory motor functions the nervous system. Nerve studies challenging conduct rodents, involvement of muscle activity makes it difficult isolate independently. This protocol provides...

10.21769/bioprotoc.5233 article EN cc-by-nc BIO-PROTOCOL 2025-01-01

ABSTRACT Background ADVANCE‐CIDP IVIG evaluated the efficacy and safety of immune globulin infusion (human) 10% solution (IVIG 10%; GAMMAGARD LIQUID, also known as Kiovig) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) a rescue treatment for patients relapsing during 1 trial. Methods Open‐label included adult with confirmed CIDP relapse (≥ 1‐point increase adjusted Inflammatory Neuropathy Cause Treatment [INCAT] disability scores from pre‐treatment baseline) 1, which...

10.1111/ene.70110 article EN cc-by-nc European Journal of Neurology 2025-04-01

The normal limits of nerve conduction studies are commonly determined by testing healthy subjects. However, in comprehensive real-life electrodiagnostic (EDX) evaluations, multiple nerves tested, including nerves, for purposes comparison with abnormal ones.This study aims to evaluate the average values a large population and examined influence age sex.EDX parameters were extracted from an electronic database performed May 2016 February 2022. Established used determine classification as...

10.3233/jnd-230052 article EN other-oa Journal of Neuromuscular Diseases 2023-08-04

Neurologic immune-related adverse events (n-irAEs) reportedly occur in up to 8% of patients treated with immune checkpoint inhibitors (ICIs) all age groups. We investigated the association between and n-irAEs ICIs examined effect on survival outcomes a large cohort melanoma.

10.1212/wnl.0000000000207632 article EN Neurology 2023-08-31

ABSTRACT Background Hereditary transthyretin (ATTRv) amyloidosis is a rare, adult‐onset autosomal‐dominant disorder caused by pathogenic variants in the ( TTR ) gene. Data about relevant specific populations and typical initial manifestations may facilitate early diagnosis treatment. We here describe genetic landscape of ATTRv Israel. Methods Genetic clinical data variant carriers patients were collected from national referral clinic other subspecialty clinics Genotype–phenotype correlations...

10.1111/ene.70057 article EN cc-by European Journal of Neurology 2025-01-29

Apolipoprotein E (APOE) ε4 gene allele and type 2 diabetes mellitus (T2DM) are prime risk factors for Alzheimer’s disease (AD). Despite evidence linking T2DM apoE4, the mechanism underlying their interaction is yet to be determined. In present study, we employed a model of APOE-targeted replacement mice high-fat diet (HFD)-induced insulin resistance investigate diabetic mechanisms associated with apoE4 pathology extent which they driven by peripheral central processes. Results obtained...

10.3390/ijms21041289 article EN International Journal of Molecular Sciences 2020-02-14

Vascular denervation occurs in some neuropathies, but measurement of small perivascular axons has been difficult.We evaluated 31 consecutive patients who had both muscle and skin biopsies. We quantitated myovascular innervation by staining unmyelinated with peripherin non-myelinating Schwann cells neural cell adhesion molecule comparing their areas.Perivascular axon-Schwann (UAS) ratios correlated axon density (r = 0.679; P < 0.0001). Low UAS (≤0.25) a sensitivity 90% specificity 91% for...

10.1002/mus.24356 article EN Muscle & Nerve 2014-08-05
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