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The rabbit has been recognized as a valuable model in various biomedical and biological research fields because of its intermediate size and phylogenetic proximity to primates. However, the technology for precise genome manipulations in rabbit has been stalled for decades, severely limiting its applications in biomedical research. Novel genome editing technologies, especially CRISPR/Cas9, have remarkably enhanced precise genome manipulation in rabbits, and shown their superiority and promise for generating rabbit models of human genetic diseases. In this review, we summarize the brief history of transgenic rabbit technology and the development of novel genome editing technologies in rabbits.We report 2 cases of patients with Parkinson's disease who exhibited bilateral vocal cord paralysis induced by an indwelling nasogastric tube (N-G tube). Both patients showed abrupt inspiratory stridor after N-G tube placement. A fiberoptic laryngeal examination revealed bilateral vocal cord abductor paralysis (VCAP). After N-G tube removal, patient symptoms improved. Nasogastric tube syndrome (NGTS) is an uncommon but life-threatening syndrome that causes sore throat and bilateral VCAP following N-G tube insertion. Throat pain is considered an important early manifestation of NGTS. However, in cases of advanced Parkinson's disease, subjective symptoms of NGTS, such as throat pain, may be difficult to recognize. We here report 2 patients with parkinson's disease accompanied by NGTS with literature review and proposed that inspiratory strider is a useful objective symptom in early diagnosing of NGTS. (Received March 25, 2020; Accepted May 18, 2020; Published September 1, 2020).A 60-year-old Japanese woman presented with left upper limb numbness and muscle weakness nine months after completing chemotherapy for diffuse large B-cell lymphoma. Magnetic resonance imaging showed distinct signal abnormalities in the left brachial plexus, but positron emission tomography/computed tomography revealed only slight linear F-fluorodeoxyglucose uptake along the involved nerves, mimicking inflammatory neuropathy. There have only been a few reports of neurolymphomatosis mimicking inflammatory neuropathy on imaging; therefore, we report the details of our case. (Received March 30, 2020; Accepted May 15, 2020; Published September 1, 2020).Although progressive multifocal leukoencephalopathy (PML) is known as a fatal disease, some recent cases have shown favorable prognosis with early diagnosis. Therefore, detection of early PML lesions using magnetic resonance imaging (MRI) is important. PML lesions are divided into 4 groups based on distinct developing patterns A)cerebral lesion, B)central lesion including deep gray matter, C)infratentorial lesion of the brain stem and cerebellum, and D)punctate lesions in the deep white matter. These lesions develop in 3 steps 1)initiation of a small demyelinating lesion, 2)extension/expansion, and 3)fusion. It is likely that the viruses first reach the brain via the bloodstream and form small demyelinating foci (initiation). Second, the demyelinating foci spread along nerve fibers or expand at the sites (extension/expansion). Finally, the foci fuse with one another to form larger demyelinating lesions (fusion). Understanding the spreading patterns of the virus could help early MRI diagnosis of PML, which is important for favorable prognosis. (Received May 7, 2020; Accepted May 11, 2020; Published September 1, 2020).Cortical cerebellar atrophy has been defined as a sporadic degenerative cerebellar disorder other than multiple system atrophy, whereas the term "idiopathic cerebellar ataxia (IDCA)" has been recently proposed by a Japanese expert group. There is no diagnostic biomarker for IDCA; therefore, the diagnosis largely depends on exclusion of other cerebellar diseases, such as multiple system atrophy and hereditary spinocerebellar ataxia. Other important differential diagnoses include immune-mediated cerebellar ataxia, such as Hashimoto's encephalopathy, gluten ataxia, anti-glutamic acid decarboxylase-positive cerebellar ataxia, alcoholic cerebellar degeneration, and drug-induced ataxia. Secondary cerebellar disorders are treatable and should be recognized and screened.Recently, the diagnostic criteria for idiopathic cerebellar ataxia (IDCA) have been proposed in Japan as a diagnosis to replace the clinical concept of cortical cerebellar atrophy, which was originally described as a neuropathological disorder. However, IDCA proposed in Japan may include various diseases such as multiple system atrophy with early stage, rare hereditary ataxias, and autoimmune-mediated cerebellar ataxia. We tackled this significant clinical challenge by detecting anti-cerebellar autoantibodies in patients' sera and identifying their target antigens. We detected anti-cerebellar autoantibodies in the sera of some patients diagnosed with IDCA in Japan. In the future, it will be necessary to confirm the efficacy of immunotherapy for anti-cerebellar autoantibody-positive cases among patients who were thought to be difficult to treat.Cortical cerebellar atrophy (CCA) contains hereditary spinocerebellar degeneration (hSCD) and genetic testing is necessary for an accurate diagnosis. Screening for frequent hSCDs (triplet repeat disease and SCA31) was performed. Panel analysis and whole exome analysis using a next-generation sequencer were also performed. The Japan Consortium for Ataxias, J-CAT, contributes to the elucidation of the genetic epidemiology of CCA. The elucidation of CCA would be promoted by comprehensive gene analysis, including whole genome analysis.Cerebellar cortical atrophy (CCA) is a neurodegenerative disease characterized by the loss of Purkinje cells, frequently associated with atrophy of the inferior olivary nucleus. The diagnosis of CCA requires a pathologic assessment; however, this term has also been used in clinical practice as a diagnosis of exclusion for sporadic, adult-onset, and progressive ataxia. For the clinical diagnosis of CCA, diagnostic criteria for idiopathic cerebellar ataxia (IDCA) have been proposed. We herein describe two patients with a pathologic diagnosis of CCA. The first patient was clinically suspected to have Creutzfeldt-Jakob disease due to rapid progressive dementia and ataxia. The second patient was clinically diagnosed with progressive supranuclear palsy based on imbalance, frequent falls, and vertical gaze palsy. The se cases suggest that the clinical presentation of CCA is heterogeneous, and CCA does not always meet the criteria for IDCA. PKI 14-22 amide,myristoylated in vivo Therefore, the term CCA should be used solely for a pathologic diagnosis.
Website: https://www.selleckchem.com/peptide/pki-14-22-amide-myristoylated.html
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