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瑞士褐牛的牛进行性退行性脑脊髓病(“韦弗病”)病变

Lesions in bovine progressive degenerative myeloencephalopathy ("Weaver") of Brown Swiss cattle.

作者信息

Stuart L D, Leipold H W

出版信息

Vet Pathol. 1985 Jan;22(1):13-23. doi: 10.1177/030098588502200103.

DOI:10.1177/030098588502200103
PMID:3976130
Abstract

Gross changes and other necropsy findings in 36 purebred Brown Swiss cattle affected with bovine progressive degenerative myeloencephalopathy were nonspecific. Primary microscopic lesions were confined to the central nervous system, specifically the white matter of the spinal cord, axons in some brainstem nuclei, and Purkinje cells of the cerebellar cortex. Spinal cord lesions involved only the white matter and consisted of axonal degeneration, loss of axons and myelin, and status spongiosus. Axonal degeneration was characterized by swelling and fragmentation of the axoplasm or formation of large, discontinuous swellings referred to as spheroids. Lesions were qualitatively similar at all levels, but quantitatively dissimilar in the same funiculi at different levels. Both ascending and descending fibers were involved but correlation to specific fasciculi was not evident. Lesions always were most severe in thoracic spinal cord segments. Little or no astroglial response, no inflammatory response, and no involvement of gray matter were observed in the spinal cord. Cerebellar lesions were limited to selective degeneration and loss of Purkinje cells and occasional swelling of Purkinje cell axons (torpedos) in the granular layer of the cerebellar cortex. Brainstem lesions were inconsistent and limited to occasional axonal swelling in brainstem nuclei. The pathogenesis of bovine progressive degenerative myeloencephalopathy is unknown and possible mechanisms were discussed. The disease exhibits a familial pattern in Brown Swiss cattle and may be hereditary. Extraneural lesions were considered secondary to central nervous system lesions.

摘要

36头患有牛进行性变性脑脊髓病的纯种瑞士褐牛的大体变化及其他尸检结果无特异性。主要的微观病变局限于中枢神经系统,具体为脊髓白质、一些脑干核中的轴突以及小脑皮质的浦肯野细胞。脊髓病变仅累及白质,包括轴突变性、轴突和髓鞘丧失以及海绵状状态。轴突变性的特征是轴浆肿胀和断裂或形成称为球体的大的、不连续的肿胀。病变在所有水平上性质相似,但在不同水平的同一脊髓索中数量不同。上行和下行纤维均受累,但与特定束的相关性不明显。病变在胸段脊髓节段总是最严重。脊髓中几乎没有或没有星形胶质细胞反应、没有炎症反应且灰质未受累。小脑病变局限于浦肯野细胞的选择性变性和丧失以及小脑皮质颗粒层中浦肯野细胞轴突偶尔肿胀(鱼雷样)。脑干病变不一致,仅限于脑干核中偶尔的轴突肿胀。牛进行性变性脑脊髓病的发病机制尚不清楚,并讨论了可能的机制。该病在瑞士褐牛中呈家族性模式,可能是遗传性的。神经外病变被认为是中枢神经系统病变的继发性病变。

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Lesions in bovine progressive degenerative myeloencephalopathy ("Weaver") of Brown Swiss cattle.瑞士褐牛的牛进行性退行性脑脊髓病(“韦弗病”)病变
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Confirmation of a non-synonymous SNP in PNPLA8 as a candidate causal mutation for Weaver syndrome in Brown Swiss cattle.确认PNPLA8中一个非同义单核苷酸多态性作为瑞士褐牛韦弗综合征的候选致病突变。
Genet Sel Evol. 2016 Mar 18;48:21. doi: 10.1186/s12711-016-0201-5.
2
Fine mapping for Weaver syndrome in Brown Swiss cattle and the identification of 41 concordant mutations across NRCAM, PNPLA8 and CTTNBP2.在瑞士褐牛中对 Weaver 综合征进行精细定位,并在 NRCAM、PNPLA8 和 CTTNBP2 中鉴定出 41 个一致突变。
PLoS One. 2013;8(3):e59251. doi: 10.1371/journal.pone.0059251. Epub 2013 Mar 20.
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Two novel cSNPs of weaver gene in Chinese indigenous goat and their associations with milk yield.
中国本土山羊中韦弗基因的两个新型cSNP及其与产奶量的关联
Mol Biol Rep. 2010 Jan;37(1):563-9. doi: 10.1007/s11033-009-9810-1.
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Bovine progressive degenerative myeloencephalopathy (weaver syndrome) in brown swiss cattle in Canada: a literature review and case report.加拿大褐牛中的牛进行性退行性脊髓脑病(韦弗综合征):文献综述与病例报告
Can Vet J. 1988 Apr;29(4):370-7.
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Microsatellite mapping of the gene causing weaver disease in cattle will allow the study of an associated quantitative trait locus.
Proc Natl Acad Sci U S A. 1993 Feb 1;90(3):1058-62. doi: 10.1073/pnas.90.3.1058.
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