Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory
Mucopolysaccharidosis I (MPS I) results from a deficiency of a lysosomal enzyme, alpha-L-iduronidase (IDUA). IDUA deficiency leads to glycosaminoglycan (GAG) accumulation resulting in cellular degeneration and multi-organ dysfunction. The primary aims of this pilot study were to determine the feasib...
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MDPI AG
2020-02-01
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| Series: | Animals |
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| Online Access: | https://www.mdpi.com/2076-2615/10/3/397 |
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| author | Lena Provoost Carlo Siracusa Darko Stefanovski Yan Che Mingyao Li Margret Casal |
| author_facet | Lena Provoost Carlo Siracusa Darko Stefanovski Yan Che Mingyao Li Margret Casal |
| author_sort | Lena Provoost |
| collection | DOAJ |
| description | Mucopolysaccharidosis I (MPS I) results from a deficiency of a lysosomal enzyme, alpha-L-iduronidase (IDUA). IDUA deficiency leads to glycosaminoglycan (GAG) accumulation resulting in cellular degeneration and multi-organ dysfunction. The primary aims of this pilot study were to determine the feasibility of cognitive testing MPS I affected dogs and to determine their non-social cognitive abilities with and without gene therapy. Fourteen dogs were tested: 5 MPS I untreated, 5 MPS I treated, and 4 clinically normal. The treated group received intrathecal gene therapy as neonates to replace the <i>IDUA</i> gene. Cognitive tests included delayed non-match to position (DNMP), two-object visual discrimination (VD), reversal learning (RL), attention oddity (AO), and two-scent discrimination (SD). Responses were recorded as correct, incorrect, or no response, and analyzed using mixed effect logistic regression analysis. Significant differences were not observed among the three groups for DNMP, VD, RL, or AO. The MPS I untreated dogs were excluded from AO testing due to failing to pass acquisition of the task, potentially representing a learning or executive function deficit. The MPS I affected group (treated and untreated) was significantly more likely to discriminate between scents than the normal group, which may be due to an age effect. The normal group was comprised of the oldest dogs, and a mixed effect logistic model indicated that older dogs were more likely to respond incorrectly on scent discrimination. Overall, this study found that cognition testing of MPS I affected dogs to be feasible. This work provides a framework to refine future cognition studies of dogs affected with diseases, including MPS I, in order to assess therapies in a more comprehensive manner. |
| first_indexed | 2024-04-13T11:36:03Z |
| format | Article |
| id | doaj.art-3763ed42f83646318c4884438d61efd5 |
| institution | Directory Open Access Journal |
| issn | 2076-2615 |
| language | English |
| last_indexed | 2024-04-13T11:36:03Z |
| publishDate | 2020-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Animals |
| spelling | doaj.art-3763ed42f83646318c4884438d61efd52022-12-22T02:48:27ZengMDPI AGAnimals2076-26152020-02-0110339710.3390/ani10030397ani10030397Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and MemoryLena Provoost0Carlo Siracusa1Darko Stefanovski2Yan Che3Mingyao Li4Margret Casal5Small Animal Behavior Service, Department of Clinical Sciences and Advanced Medicine, University of Pennsylvania, 3900 Spruce Street, Philadelphia, PA 19104, USASmall Animal Behavior Service, Department of Clinical Sciences and Advanced Medicine, University of Pennsylvania, 3900 Spruce Street, Philadelphia, PA 19104, USADepartment of Clinical Sciences and Advanced Medicine, University of Pennsylvania, New Bolton Center, Kennett Square, PA 19348, USAGene Therapy Program, Department of Medicine, The Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Blvd., Philadelphia, PA 19104, USADepartment of Biostatistics and Epidemiology, The Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Blvd., Philadelphia, PA 19104, USASection of Medical Genetics, Department of Clinical Sciences and Advanced Medicine, University of Pennsylvania, 3900 Spruce Street, Philadelphia, PA 19104, USAMucopolysaccharidosis I (MPS I) results from a deficiency of a lysosomal enzyme, alpha-L-iduronidase (IDUA). IDUA deficiency leads to glycosaminoglycan (GAG) accumulation resulting in cellular degeneration and multi-organ dysfunction. The primary aims of this pilot study were to determine the feasibility of cognitive testing MPS I affected dogs and to determine their non-social cognitive abilities with and without gene therapy. Fourteen dogs were tested: 5 MPS I untreated, 5 MPS I treated, and 4 clinically normal. The treated group received intrathecal gene therapy as neonates to replace the <i>IDUA</i> gene. Cognitive tests included delayed non-match to position (DNMP), two-object visual discrimination (VD), reversal learning (RL), attention oddity (AO), and two-scent discrimination (SD). Responses were recorded as correct, incorrect, or no response, and analyzed using mixed effect logistic regression analysis. Significant differences were not observed among the three groups for DNMP, VD, RL, or AO. The MPS I untreated dogs were excluded from AO testing due to failing to pass acquisition of the task, potentially representing a learning or executive function deficit. The MPS I affected group (treated and untreated) was significantly more likely to discriminate between scents than the normal group, which may be due to an age effect. The normal group was comprised of the oldest dogs, and a mixed effect logistic model indicated that older dogs were more likely to respond incorrectly on scent discrimination. Overall, this study found that cognition testing of MPS I affected dogs to be feasible. This work provides a framework to refine future cognition studies of dogs affected with diseases, including MPS I, in order to assess therapies in a more comprehensive manner.https://www.mdpi.com/2076-2615/10/3/397mps idogcognitionmemorylearning |
| spellingShingle | Lena Provoost Carlo Siracusa Darko Stefanovski Yan Che Mingyao Li Margret Casal Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory Animals mps i dog cognition memory learning |
| title | Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory |
| title_full | Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory |
| title_fullStr | Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory |
| title_full_unstemmed | Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory |
| title_short | Cognitive Abilities of Dogs with Mucopolysaccharidosis I: Learning and Memory |
| title_sort | cognitive abilities of dogs with mucopolysaccharidosis i learning and memory |
| topic | mps i dog cognition memory learning |
| url | https://www.mdpi.com/2076-2615/10/3/397 |
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