Möller J R, McLenigan M, Potts B J, Quarles R H
Demyelinating Disorders Unit, LMCN, NINDS, NIH, Bethesda, MD 20892.
J Neurochem. 1993 Nov;61(5):1808-12. doi: 10.1111/j.1471-4159.1993.tb09820.x.
Border disease (BD) of sheep is caused by a virus in the genus Pestivirus that results in decreased myelination throughout the CNS when acquired congenitally. Pregnant ewes were inoculated with BD virus at 50 days of gestation, and myelin proteins were quantified in several regions of the CNS during prenatal and postnatal development of infected lambs for comparison with age-matched controls. Newborn field-infected lambs were also examined. Myelin basic protein (MBP), proteolipid protein (PLP), myelin-associated glycoprotein (MAG), and 2',3'-cyclic nucleotide 3'-phosphodiesterase (CNP) were measured by densitometric scanning of western blots. Deficiencies in the myelin proteins were detected as early as 116 days of gestation, and the deficiencies of myelin proteins were most pronounced in the cerebellum at all ages examined. PLP and MBP increased from 10-30% of normal in cerebellar white matter at birth to 40-60% of normal at 6 months, suggesting some catch-up in the amount of compact myelin with development. MAG and CNP were between 70 and 80% of control levels in the cerebellum at birth and at 6 months. Similar results were obtained for the corpus callosum and spinal cord of infected lambs, but the deficiencies of myelin proteins were not as great. A common finding in all regions examined was that MBP and PLP were reduced more than MAG and CNP. This is probably explained by a greater deficit of compact myelin, in which MBP and PLP are localized, than of associated oligodendroglial membranes, in which MAG and CNP are concentrated.(ABSTRACT TRUNCATED AT 250 WORDS)
绵羊边境病（BD）由瘟病毒属中的一种病毒引起，先天性感染该病毒会导致整个中枢神经系统（CNS）的髓鞘形成减少。在妊娠50天时给怀孕母羊接种BD病毒，并在受感染羔羊的产前和产后发育期间对CNS的几个区域的髓鞘蛋白进行定量，以与年龄匹配的对照进行比较。还检查了新生的野外感染羔羊。通过蛋白质印迹的光密度扫描测量髓鞘碱性蛋白（MBP）、蛋白脂蛋白（PLP）、髓鞘相关糖蛋白（MAG）和2'，3'-环核苷酸3'-磷酸二酯酶（CNP）。早在妊娠116天时就检测到髓鞘蛋白缺乏，并且在所检查的所有年龄段中，小脑的髓鞘蛋白缺乏最为明显。PLP和MBP从小脑白质出生时正常水平的10-30%增加到6个月时正常水平的40-60%，表明随着发育，紧密髓鞘的量有所恢复。出生时和6个月时，小脑的MAG和CNP为对照水平的70-80%。在受感染羔羊的胼胝体和脊髓中也获得了类似的结果，但髓鞘蛋白的缺乏程度没有那么严重。在所有检查区域中一个常见的发现是，MBP和PLP的减少比MAG和CNP更多。这可能是因为与MAG和CNP集中的相关少突胶质细胞膜相比，MBP和PLP所在的紧密髓鞘的缺陷更大。（摘要截断于250字）
