the syndrome of arthrogryposis and palatoschisis (sap) in charolais cattle; abnormal motor innervation and defect in the focalization of 16 s acetylcholinesterase in the end-plates rich regions of the muscle关节弯曲的综合症,腭裂(sap)charolais牛;.pdfVIP
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the syndrome of arthrogryposis and palatoschisis (sap) in charolais cattle; abnormal motor innervation and defect in the focalization of 16 s acetylcholinesterase in the end-plates rich regions of the muscle关节弯曲的综合症,腭裂(sap)charolais牛;
The Syndrome of Arthrogryposis and Palatoschisis (SAP)
in Charolais cattle; Abnormal motor innervation and defect
in the focalization of 16 S acetylcholinesterase in the end-
plates rich regions of the muscle. (1)
F. RIEGER, Martine PINÇON-RAYMOND, P. DREYFUS
Monique GUITTARD M. FARDEAU
Groupe de Biologie et Pathologie Neuromusculaives I.N.S.E.R.M. U 153.
17,rue du Fey-à-Moulin, oogq$Paris, France
Summary
The Syndrome of Arthrogryposis and Palatoschisis SAP is an hereditary disease in the
Charolais cattle bread. We studied the neurobiological and neuropathological correlates of
this disease in SAP calves, immediately after birth. We find that nerve-muscle interaction is
abnormal; the focal accumulation of 6S1AChE in end-plate rich regions is not observed as in
control calf, and the motor innervation, evidenced by silver nitrate impregnation techniques,
shows abnormal features, as frequent preterminal branching and ultraterminal sprouting.
Introduction
The Syndrome of Arthrogryposis and Palatoschisis (SAP) in Charolais cattle
has been first described and It is an
by AUVERGNEL UNB19).67( hereditary
disease, carried by an autosomal recessive gene with incomplete penetrance (see
EFORTaIet al. 1977 and also AUVNGRLEEand AUrr,OCF1976, and LAUVERGNE
et al. 1979 for a review).
Its clinical expression is rather variable in severity, but is always characterized
by fixture of joints (arthrogryposis) and severity of the disease is the consequence
of different movement disabilities of the limbs, due to variable angles of fixture
and also aberrant insertion of muscles or even lack of a given muscle. In man,
arthrogryposis has been described as a congenital
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