Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis i (MPS I)

Louise D. Archer; Kia J. Langford-Smith; William R. Critchley; Brian W. Bigger; James E. Fildes

doi:10.1007/s10545-012-9508-8

Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis i (MPS I)

Louise D. Archer, Kia J. Langford-Smith, William R. Critchley, Brian W. Bigger, James E. Fildes

Research output: Contribution to journal › Article › peer-review

Abstract

Background: Mucopolysaccharidosis I (MPS I) is a metabolic disorder caused by α-L-Iduronidase (IDUA) deficiency, resulting in lysosomal accumulation of heparan (HS) and dermatan sulphate (DS). This has been reported in microglia, yet currently the effect of IDUA deficiency on T cells and dendritic cells (DC) and their functionality in disease pathogenesis remains unclear. Methods: Peripheral blood was collected from 3 month old C57BL/6 MPS I (n = 11) and wildtype (WT) (n = 6) mice. T cell and DC phenotype and functional characteristics were identified by flow cytometry. Results: MPS I mice exhibited a reduction in DC (p =

Original language	English
Pages (from-to)	257-262
Number of pages	5
Journal	Journal of Inherited Metabolic Disease
Volume	36
Issue number	2
DOIs	https://doi.org/10.1007/s10545-012-9508-8
Publication status	Published - Mar 2013

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title = "Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis i (MPS I)",

abstract = "Background: Mucopolysaccharidosis I (MPS I) is a metabolic disorder caused by α-L-Iduronidase (IDUA) deficiency, resulting in lysosomal accumulation of heparan (HS) and dermatan sulphate (DS). This has been reported in microglia, yet currently the effect of IDUA deficiency on T cells and dendritic cells (DC) and their functionality in disease pathogenesis remains unclear. Methods: Peripheral blood was collected from 3 month old C57BL/6 MPS I (n = 11) and wildtype (WT) (n = 6) mice. T cell and DC phenotype and functional characteristics were identified by flow cytometry. Results: MPS I mice exhibited a reduction in DC (p =",

author = "Archer, {Louise D.} and Langford-Smith, {Kia J.} and Critchley, {William R.} and Bigger, {Brian W.} and Fildes, {James E.}",

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language = "English",

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T1 - Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis i (MPS I)

AU - Archer, Louise D.

AU - Langford-Smith, Kia J.

AU - Critchley, William R.

AU - Bigger, Brian W.

AU - Fildes, James E.

PY - 2013/3

Y1 - 2013/3

N2 - Background: Mucopolysaccharidosis I (MPS I) is a metabolic disorder caused by α-L-Iduronidase (IDUA) deficiency, resulting in lysosomal accumulation of heparan (HS) and dermatan sulphate (DS). This has been reported in microglia, yet currently the effect of IDUA deficiency on T cells and dendritic cells (DC) and their functionality in disease pathogenesis remains unclear. Methods: Peripheral blood was collected from 3 month old C57BL/6 MPS I (n = 11) and wildtype (WT) (n = 6) mice. T cell and DC phenotype and functional characteristics were identified by flow cytometry. Results: MPS I mice exhibited a reduction in DC (p =

AB - Background: Mucopolysaccharidosis I (MPS I) is a metabolic disorder caused by α-L-Iduronidase (IDUA) deficiency, resulting in lysosomal accumulation of heparan (HS) and dermatan sulphate (DS). This has been reported in microglia, yet currently the effect of IDUA deficiency on T cells and dendritic cells (DC) and their functionality in disease pathogenesis remains unclear. Methods: Peripheral blood was collected from 3 month old C57BL/6 MPS I (n = 11) and wildtype (WT) (n = 6) mice. T cell and DC phenotype and functional characteristics were identified by flow cytometry. Results: MPS I mice exhibited a reduction in DC (p =

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DO - 10.1007/s10545-012-9508-8

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C2 - 22773246

SN - 0141-8955

VL - 36

SP - 257

EP - 262

JO - Journal of Inherited Metabolic Disease

JF - Journal of Inherited Metabolic Disease

IS - 2

ER -

Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis i (MPS I)

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