Vitamin C Depletion in Prenatal Guinea Pigs as a Model of Lissencephaly Type II

Humans and guinea pigs are unable to produce vitamin C, with deficiency resulting in a well-known disorder of collagen synthesis. Pial basement membrane structure preservation is essential in the proper migration of neurons. In our study, intrauterine deprivation of vitamin C in guinea pig fetuses l...

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Bibliographic Details
Published in:Veterinary pathology 2015-11, Vol.52 (6), p.1263-1271
Main Authors: Čapo, I., Hinić, N., Lalošević, D., Vučković, N., Stilinović, N., Marković, J., Sekulić, S.
Format: Article
Language:English
Subjects:
Animals
Ascorbic Acid Deficiency - pathology
Ascorbic Acid Deficiency - veterinary
Basement Membrane - pathology
Cerebellum - pathology
Collagen - metabolism
Disease Models, Animal
Female
Guinea Pigs
Humans
Lissencephaly - pathology
Lissencephaly - veterinary
Male
Neurons - pathology
Scurvy - pathology
Scurvy - veterinary
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Summary:Humans and guinea pigs are unable to produce vitamin C, with deficiency resulting in a well-known disorder of collagen synthesis. Pial basement membrane structure preservation is essential in the proper migration of neurons. In our study, intrauterine deprivation of vitamin C in guinea pig fetuses led to a collagen synthesis disorder, weakness, and finally a breach of pial basement membrane. We found excessive migration of the external germinal layer cells into the subarachnoid space of the cerebellum through defects in the pial basement membrane. The changes ranged from focal rupture of pial basement membranes to their complete disintegration. The loss of proper folia formation resulted in macroscopically visible flattening of the cerebellar surface. Different grades of dysplastic changes in the folia of the cerebellar cortex were observed in 2 experimental groups assigned different limits to mark the time of commencement and duration of vitamin C deprivation. The most severe form of dysplastic changes was characterized by marked irregularity of the cerebellar cortex similar to that in lissencephaly type II. Thus, prenatal vitamin C deficiency represents a novel animal model to study the effects of collagen synthesis on development of breaches in the pial basement membrane, disordered migration of neurons, dysplasia of cerebellar cortex, and the pathogenesis of lissencephaly.
ISSN:0300-9858
1544-2217
DOI:10.1177/0300985814561270