title: Analysis of male reproductive parameters in a murine model of mucopolysaccharidosis type I (MPS I).
abstract: Mucopolysaccharidosis (MPS) I is a lysosomal storage disorder (LSD) that is characterised by alpha-L-iduronidase (Idua) deficiency and continuous deposition of glycosaminoglycans (GAGs), which consequently interferes with cell signalling mechanisms and results in multisystemic and progressive symptoms. The animal model of MPS I (Idua-/-) has been widely studied to elucidate the consequences and progression of the disorder; however, studies specifically assessing the male reproductive tract are lacking. The aim of this study was to evaluate some of the reproductive characteristics of male MPS I mice in two phases of life. Reproductive organ biometry, sperm counts, sperm morphological evaluation, plasma testosterone measurements and histopathological, histomorphometrical and immunohistochemical analysis were performed in 3- and 6-month-old C57BL/6 Idua+/+ and Idua-/- mice. Seminal vesicle weights were decreased in both the 3- and 6-month-old Idua-/- mice. Decrease in sperm counts and the majority of the histopathological signs were observed in the 6-month-old Idua-/- mice. No differences were detected in the sperm morphological analysis. Immunohistochemistry revealed that seminiferous tubules from 3-month-old Idua-/- mice were more intensely stained with anti-caspase-3 than 3-month-old Idua+/+ mice, but no difference was found at 6 months. These results suggest that MPS I interferes with male reproductive parameters both in 3 and 6-month-old animals and histopathological signs are more pronounced in 6-month-old mice, indicating that the effects of the disorder may intensify with the disease progression.
authors.affiliation: Department of Psychobiology, Universidade Federal de São Paulo São Paulo - SP, Brazil.
authors.forename: Cinthia Castro
authors.initials: CC
authors.lastname: do Nascimento
authors.affiliation: Department of Biosciences, Universidade Federal de São Paulo Santos - SP, Brazil.
authors.forename: Odair Aguiar
authors.initials: OA
authors.lastname: Junior
authors.affiliation: Department of Psychobiology, Universidade Federal de São Paulo São Paulo - SP, Brazil.
authors.forename: Vânia
authors.initials: V
authors.lastname: D'Almeida
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D018450:Disease Progression; D005832:Genital Diseases, Male / Q000209:etiology* / Q000473:pathology*; D005837:Genitalia, Male / Q000473:pathology*; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D008059:Mucopolysaccharidosis I / Q000150:complications*; D013076:Sperm Count; D013094:Spermatozoa / Q000473:pathology; D013739:Testosterone / Q000097:blood
chemical_list: D013739:Testosterone
keywords: Knockout mice; mucopolysaccharidosis; spermatogenesis; testis