کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8684707 | 1580137 | 2018 | 12 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Mice overexpressing lamin B1 in oligodendrocytes recapitulate the age-dependent motor signs, but not the early autonomic cardiovascular dysfunction of autosomal-dominant leukodystrophy (ADLD)
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کلمات کلیدی
PBSPLP1SYMLMNB1ADLDTTOTRSABRSElectroencephalogram - الکتروانسفالوگرافیEMG - الکترومیوگرافیelectromyogram - الکترومیوگرافیOligodendrocyte - الیگودندروسیتMinute volume - حجم دقیقهTidal volume - حجم نزولیSleep - خوابnon-rapid-eye-movement sleep - خواب بدون حرکت سریع چشمRapid-eye-movement sleep - خواب سریع سریع چشمSympathetic - دلسوزHeart period - دوره قلبgait - راه رفتنSAP - شیرهHeart rate - ضربان قلبPhosphate buffered saline - فسفات بافر شورarterial pressure - فشار شریانsystolic arterial pressure - فشار شریانی سیستولیکLamin - لامینLeukodystrophy - لکوودیستروفیMice - موشBreathing - نفس کشیدنEEG - نوار مغزیproteolipid protein 1 - پروتئولپید پروتئین 1
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
عصب شناسی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Autosomal dominant leukodystrophy (ADLD) is a rare adult-onset demyelinating disease caused by overexpression of lamin B1, a nuclear lamina filament. Early autonomic dysfunction involving the cardiovascular system before progressive somatic motor dysfunction is a striking feature of most cases of ADLD. In the Plp-FLAG-LMNB1 transgenic mouse model, lamin B1 overexpression in oligodendrocytes elicits somatic motor dysfunction and neuropathology akin to ADLD. Here, we investigate whether Plp-FLAG-LMNB1 mice also develop autonomic cardiovascular dysfunction before or after somatic motor dysfunction. We find that Plp-FLAG-LMNB1 mice have preserved cardiovascular responses to changes in wake-sleep state and ambient temperature and normal indexes of autonomic modulation at 37-42Â weeks of age despite a progressive somatic motor dysfunction, which includes impairments of walking ability (the ability to walk on a narrow path was impaired in 80% of mice at 34-38Â weeks of age) and subtle breathing derangements. Only late in the development of the disease phenotype did Plp-FLAG-LMNB1 mice develop a structural deficit of sympathetic noradrenergic fibers, with a 38% decrease in fiber profiles in the kidneys at 44-47Â weeks of age. We demonstrate that while the Plp-FLAG-LMNB1 mouse model recapitulates the age-dependent motor dysfunction of ADLD, it does not show signs of early autonomic cardiovascular dysfunction, raising the possibility that oligodendrocyte dysfunction may not be sufficient to cause the full spectrum of clinical features present in ADLD.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Experimental Neurology - Volume 301, Part A, March 2018, Pages 1-12
Journal: Experimental Neurology - Volume 301, Part A, March 2018, Pages 1-12
نویسندگان
Viviana Lo Martire, Sara Alvente, Stefano Bastianini, Chiara Berteotti, Cristiano Bombardi, Giovanna Calandra-Buonaura, Sabina Capellari, Gary Cohen, Pietro Cortelli, Laura Gasparini, Quasar Padiath, Alice Valli, Giovanna Zoccoli, Alessandro Silvani,