Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse

被引：53

作者：

Rathke-Hartlieb, S ^{[1
]}

Schmidt, VC ^{[1
]}

Jockusch, H ^{[1
]}

Schmitt-John, T ^{[1
]}

Bartsch, JW ^{[1
]}

机构：

[1] Univ Bielefeld, D-33501 Bielefeld, Germany

来源：

NEUROREPORT | 1999年 / 10卷 / 16期

关键词：

genetic diagnosis; glia activation; neurodegeneration; wobbler mouse;

D O I：

10.1097/00001756-199911080-00028

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

THE wobbler mouse (phenotype WR; genotype wr/wr) has been investigated as a model for neurodegenerative diseases like SMA and ALS. A new; diagnostic market based on a polymorphism in the closely linked chaperonine gene Cct4 enabled us to diagnose the allelic status at the wr locus within the original background-strain C57BL/6. Using this marker, we investigated the spatiotemporal progression of neuropathology in WR mice from postnatal day (d.p.n.) 10 to 60. Neurodegeneration starts at 13 d.p.n. in the thalamus (N. ventralis), in deep cerebellar nuclei, brain stem (N. vestibularis) and spinal cord interneurons. The motor nuclei of spinal nerves and motoneurons degenerate from 15 d.p.n. onward. Reactive astrocytes are observed around 17 d.p.n. in the white and grey matter of the spinal cord. Microgliosis occurs only from 23 d.p.n. onward. Our data demonstrate that in the WR disease, neurodegeneration in thalamus, cerebellum and brain stem precedes motoneuron degeneration, astrogliosis and;microgliosis. NeuroReport 10:3411-3416 (C) 1999 Lippincott Williams & Wilkins.

引用

页码：3411 / 3416

页数：6

共 20 条

[1]

Augustin M, 1997, DEV DYNAM, V209, P286, DOI 10.1002/(SICI)1097-0177(199707)209:3<286::AID-AJA4>3.0.CO

[2]

2-E

[3] Increase in fiber density for immunoreactive serotonin, substance P, enkephalin and thyrotropin-releasing hormone occurs during the early presymptomatic period of motoneuron disease in Wobbler mouse spinal cord ventral horn [J].