Morphopathological features in tissues of α-mannosidosis guinea pigs at different gestational ages

D. Auclair, J. J. Hopwood

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Alpha-mannosidosis is an inherited metabolic disorder characterized by a reduction in α-D-mannosidase and intralysosomal accumulation of undegraded mannose-containing oligosaccharides. The α-mannosidosis guinea pig exhibits pathological similarities to its human counterpart, which make it a valuable animal model. To trace the progression of α-mannosidosis during foetal development, brain and visceral organs from affected and unaffected guinea pigs at 30, 36, 38, 51 and 65 days of gestation (dg) were examined by light and electron microscopy (term: approximately 68 dg). In the affected brain, distended lysosomes (vacuoles) were scarce up to 38 dg and were seen in few differentiating neuronal cells but mostly in macrophages, pericytes and endothelial cells. At 51 and 65 dg, several vacuoles were observed in some neurones, in many Purkinje cells, pericytes, endothelial and microglial cells, and in few cerebellar internal granule cells. Myelination had started by 51 dg. Non-myelinated axonal spheroids were detected in the brainstem at 65 dg. In the kidney cortex and liver, an increase in vacuolation was noticed between 36 and 65 dg. Some vacuolated cells were also noticed in the lungs and spleen at 51 and 65 dg. Altogether, these histological observations suggest that α-mannosidosis is unlikely to affect ontogenesis before the second half of gestation in guinea pigs; however, the morphopathological features recorded during the last quarter of gestation (which may roughly correspond to the period covering near term to 1-2 years of age in human) were clearly noticeable and may have had some impact.

LanguageEnglish
Pages572-585
Number of pages14
JournalNeuropathology and Applied Neurobiology
Volume33
Issue number5
DOIs
Publication statusPublished - 1 Oct 2007

Keywords

  • Alpha-mannosidosis
  • Brain
  • Foetal development
  • Guinea pigs
  • Lysosomal storage diseases
  • Vacuoles

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Neurology
  • Clinical Neurology
  • Physiology (medical)

Cite this

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abstract = "Alpha-mannosidosis is an inherited metabolic disorder characterized by a reduction in α-D-mannosidase and intralysosomal accumulation of undegraded mannose-containing oligosaccharides. The α-mannosidosis guinea pig exhibits pathological similarities to its human counterpart, which make it a valuable animal model. To trace the progression of α-mannosidosis during foetal development, brain and visceral organs from affected and unaffected guinea pigs at 30, 36, 38, 51 and 65 days of gestation (dg) were examined by light and electron microscopy (term: approximately 68 dg). In the affected brain, distended lysosomes (vacuoles) were scarce up to 38 dg and were seen in few differentiating neuronal cells but mostly in macrophages, pericytes and endothelial cells. At 51 and 65 dg, several vacuoles were observed in some neurones, in many Purkinje cells, pericytes, endothelial and microglial cells, and in few cerebellar internal granule cells. Myelination had started by 51 dg. Non-myelinated axonal spheroids were detected in the brainstem at 65 dg. In the kidney cortex and liver, an increase in vacuolation was noticed between 36 and 65 dg. Some vacuolated cells were also noticed in the lungs and spleen at 51 and 65 dg. Altogether, these histological observations suggest that α-mannosidosis is unlikely to affect ontogenesis before the second half of gestation in guinea pigs; however, the morphopathological features recorded during the last quarter of gestation (which may roughly correspond to the period covering near term to 1-2 years of age in human) were clearly noticeable and may have had some impact.",
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Morphopathological features in tissues of α-mannosidosis guinea pigs at different gestational ages. / Auclair, D.; Hopwood, J. J.

In: Neuropathology and Applied Neurobiology, Vol. 33, No. 5, 01.10.2007, p. 572-585.

Research output: Contribution to journalArticle

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