A NEW MOUSE MUTANT WITH PROGRESSIVE MOTOR NEURONOPATHY

被引:150
作者
SCHMALBRUCH, H [1 ]
JENSEN, HJS [1 ]
BJAERG, M [1 ]
KAMIENIECKA, Z [1 ]
KURLAND, L [1 ]
机构
[1] UNIV COPENHAGEN,PANUM INST,DEPT ANIM,DK-2200 COPENHAGEN,DENMARK
关键词
DYING-BACK NEUROPATHY; EXPERIMENTAL MODEL; MOTOR NEURON DISEASE; MOUSE MUTANT; PROGRESSIVE MOTOR NEURONOPATHY;
D O I
10.1097/00005072-199105000-00002
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
A new autosomal-recessive mouse mutant with progressive motor neuronopathy (pmn) is described. Homozygotes develop paralysis of the hindlimbs during the third week of life. Soon thereafter the forelimbs also become weak, and all mice die six to seven weeks after birth. Heterozygotes are normal. Skeletal muscles show neurogenic atrophy without histological signs of reinnervation. Axonal degeneration apparently starts at the endplates and is prominent in the sciatic nerve and its branches and the phrenic nerve. Axonal sprouts are abundant. There is no evidence of demyelination, and unaffected nerve fibers are normally myelinated. Sensory axons are spared. Almost all distal motor axons have disappeared in four to five weeks after birth. Ventral nerve roots show a reduced diameter of the largest fibers but no fiber deficits. The ventral horn cells show slight chromatolysis. The corticospinal tract is normal, but in terminally ill animals the fasciculus gracilis, the rubrospinal tract and possibly also reticulospinal fibers degenerate. The brain is histologically normal. The disease manifests itself in a dying-back fashion in the distal portion of the motor neurons and may represent an animal model of hereditary motor neuron diseases in man.
引用
收藏
页码:192 / 204
页数:13
相关论文
共 34 条
[1]  
Andrews J.M., 1976, AMYOTROPHIC LATERAL, P181
[2]  
BJAERG M, 1990, J PHYSIOL-LONDON, V423, pP82
[3]   NERVE AND MUSCLE DEVELOPMENT IN PARALYZE MUTANT MICE [J].
BLONDET, B ;
DUXSON, MJ ;
HARRIS, AJ ;
MELKI, J ;
GUENET, JL ;
PINCONRAYMOND, M ;
RIEGER, F .
DEVELOPMENTAL BIOLOGY, 1989, 132 (01) :153-166
[4]   MORPHOMETRIC AND BIOCHEMICAL-STUDIES OF PERIPHERAL-NERVES IN AMYOTROPHIC LATERAL SCLEROSIS [J].
BRADLEY, WG ;
GOOD, P ;
RASOOL, CG ;
ADELMAN, LS .
ANNALS OF NEUROLOGY, 1983, 14 (03) :267-277
[5]   RUBROSPINAL PROJECTIONS IN RAT [J].
BROWN, LT .
JOURNAL OF COMPARATIVE NEUROLOGY, 1974, 154 (02) :169-187
[6]  
CARPENTER S, 1975, NEUROLOGY, V25, P364
[7]  
CORK LC, 1989, LAB INVEST, V61, P69
[8]   AN HEREDITARY MOTOR NEURONE DISEASE WITH PROGRESSIVE DENERVATION OF MUSCLE IN MOUSE - MUTANTWOBBLER [J].
DUCHEN, LW ;
STRICH, SJ .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1968, 31 (06) :535-&
[10]  
DUCHEN LW, 1983, J PHYSIOL-LONDON, V345, pP166