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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2009.1
- 수록면
- 140 - 150 (11page)
이용수
초록· 키워드
오류제보하기
A subset of patients of amyotrophic lateral sclerosis
(ALS) present with mutation of Cu/Zn superoxide dismutase
1 (SOD1), and such mutants caused an ALSlike
disorder when expressed in rodents. These findings
implicated SOD1 in ALS pathogenesis and made
the transgenic animals a widely used ALS model.
However, previous studies of these animals have focused
largely on motor neuron damage. We report
herein that the spinal cords of mice expressing a human
SOD1 mutant (hSOD1-G93A), besides showing
typical destruction of motor neurons and axons, exhibit
significant damage in the sensory system, including
Wallerian-like degeneration in axons of dorsal root
and dorsal funiculus, and mitochondrial damage in
dorsal root ganglia neurons. Thus, hSOD1-G93A mutation
causes both motor and sensory neuropathies, and
as such the disease developed in the transgenic mice
very closely resembles human ALS.
목차
등록된 정보가 없습니다.
참고문헌 (22)
참고문헌 신청
Andersen PM / 2006 / Amyotrophic lateral sclerosis genetics with Mendelian inheritance / Informa Healthcare : 187 ~ 207
Dyck PJ / 1975 / Frequency of nerve fiber degeneration of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. Morphometry of deep and superficial peroneal
Feeney SJ / 2001 / Presymptomatic motor neuron loss and reactive astrocytosis in the SOD1 mouse model of amyotrophic lateral sclerosis / Muscle Nerve 24 : 1510 ~ 1519
Fischer LR / 2004 / Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man / Exp Neurol 185 : 232 ~ 240
Fischer LR / 2005 / The WldS gene modestly prolongs survival in the SOD1G93A fALS mouse / Neurobiol Dis 19 : 293 ~ 300
Dyck PJ / 1975 / Frequency of nerve fiber degeneration of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. Morphometry of deep and superficial peroneal
Feeney SJ / 2001 / Presymptomatic motor neuron loss and reactive astrocytosis in the SOD1 mouse model of amyotrophic lateral sclerosis / Muscle Nerve 24 : 1510 ~ 1519
Fischer LR / 2004 / Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man / Exp Neurol 185 : 232 ~ 240
Fischer LR / 2005 / The WldS gene modestly prolongs survival in the SOD1G93A fALS mouse / Neurobiol Dis 19 : 293 ~ 300
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The Virulence of Vibrio vulnificus is Affected by the Cellular Level ofSuperoxide Dismutase Activity
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Characteristics of the Diamond Thin Film as the SOD Structure
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