ALTERATIONS IN SYNAPTIC CONNECTIVITY IN CEREBRAL CORTEX IN NEURONAL STORAGE DISORDERS.
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| Title: | ALTERATIONS IN SYNAPTIC CONNECTIVITY IN CEREBRAL CORTEX IN NEURONAL STORAGE DISORDERS. |
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| Authors: | Walkley, Steven U., Wurzelmann, Sarah |
| Source: | Mental Retardation & Developmental Disabilities Research Reviews. 1995, Vol. 1 Issue 3, p183-192. 10p. |
| Subjects: | Neurotransmitters, Cerebral cortex, Developmental disabilities, Metabolic disorders, Neurological errors, Nervous system |
| Abstract: | Neuronal storage diseases are inborn errors of metabolism characterized by widespread intraneuronal storage and progressive neurological dysfunction, including mental retardation, movement disorders, and seizures. Most of these disorders are associated with specific defects in particular lysosomal enzymes. Moreover, a wide array of genetic defects has been documented. The immense progress made during the past three decades in understanding the primary molecular and enzymatic defects in this family of diseases stands in sharp contrast to the modest advances made in delineating pathogenetic mechanisms and treatment strategies. Although these disorders result from highly selective defects in metabolic pathways, they ultimately are complex conditions characterized by a plethora of seemingly unrelated secondary molecular and cellular abnormalities. Studies of children and animals affected by these diseases have revealed that significant changes in neuronal connectivity occur in the cerebral cortex. These include not only degenerative changes in axons and synapses of inhibitory neurons, but also a regrowth of dendrites and new synapse formation involving pyramidal neurons. The latter finding indicates that neurons in the cerebral cortex undergo significant "rewiring" during the height of the disease process. This regrowth of primary dendrites and formation of new synapses as part of a neurological disease is unprecedented. Under- standing the causes and consequences of these changes in cortical synaptic connectivity in neuronal storage diseases may provide insight not only into the precise mechanisms underlying mental retardation in these diseases and their reversibility, but also into processes governing dendritic elaboration and synapse formation in the normal developing nervous system. [ABSTRACT FROM AUTHOR] |
| Copyright of Mental Retardation & Developmental Disabilities Research Reviews is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | Psychology and Behavioral Sciences Collection |
| FullText | Links: – Type: pdflink Text: Availability: 0 |
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| Header | DbId: pbh DbLabel: Psychology and Behavioral Sciences Collection An: 12286500 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: ALTERATIONS IN SYNAPTIC CONNECTIVITY IN CEREBRAL CORTEX IN NEURONAL STORAGE DISORDERS. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Walkley%2C+Steven+U%2E%22">Walkley, Steven U.</searchLink><br /><searchLink fieldCode="AR" term="%22Wurzelmann%2C+Sarah%22">Wurzelmann, Sarah</searchLink> – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Mental+Retardation+%26+Developmental+Disabilities+Research+Reviews%22">Mental Retardation & Developmental Disabilities Research Reviews</searchLink>. 1995, Vol. 1 Issue 3, p183-192. 10p. – Name: Subject Label: Subjects Group: Su Data: <searchLink fieldCode="DE" term="%22Neurotransmitters%22">Neurotransmitters</searchLink><br /><searchLink fieldCode="DE" term="%22Cerebral+cortex%22">Cerebral cortex</searchLink><br /><searchLink fieldCode="DE" term="%22Developmental+disabilities%22">Developmental disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Metabolic+disorders%22">Metabolic disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Neurological+errors%22">Neurological errors</searchLink><br /><searchLink fieldCode="DE" term="%22Nervous+system%22">Nervous system</searchLink> – Name: Abstract Label: Abstract Group: Ab Data: Neuronal storage diseases are inborn errors of metabolism characterized by widespread intraneuronal storage and progressive neurological dysfunction, including mental retardation, movement disorders, and seizures. Most of these disorders are associated with specific defects in particular lysosomal enzymes. Moreover, a wide array of genetic defects has been documented. The immense progress made during the past three decades in understanding the primary molecular and enzymatic defects in this family of diseases stands in sharp contrast to the modest advances made in delineating pathogenetic mechanisms and treatment strategies. Although these disorders result from highly selective defects in metabolic pathways, they ultimately are complex conditions characterized by a plethora of seemingly unrelated secondary molecular and cellular abnormalities. Studies of children and animals affected by these diseases have revealed that significant changes in neuronal connectivity occur in the cerebral cortex. These include not only degenerative changes in axons and synapses of inhibitory neurons, but also a regrowth of dendrites and new synapse formation involving pyramidal neurons. The latter finding indicates that neurons in the cerebral cortex undergo significant "rewiring" during the height of the disease process. This regrowth of primary dendrites and formation of new synapses as part of a neurological disease is unprecedented. Under- standing the causes and consequences of these changes in cortical synaptic connectivity in neuronal storage diseases may provide insight not only into the precise mechanisms underlying mental retardation in these diseases and their reversibility, but also into processes governing dendritic elaboration and synapse formation in the normal developing nervous system. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Mental Retardation & Developmental Disabilities Research Reviews is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1002/mrdd.1410010306 Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 10 StartPage: 183 Subjects: – SubjectFull: Neurotransmitters Type: general – SubjectFull: Cerebral cortex Type: general – SubjectFull: Developmental disabilities Type: general – SubjectFull: Metabolic disorders Type: general – SubjectFull: Neurological errors Type: general – SubjectFull: Nervous system Type: general Titles: – TitleFull: ALTERATIONS IN SYNAPTIC CONNECTIVITY IN CEREBRAL CORTEX IN NEURONAL STORAGE DISORDERS. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Walkley, Steven U. – PersonEntity: Name: NameFull: Wurzelmann, Sarah IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 08 Text: 1995 Type: published Y: 1995 Identifiers: – Type: issn-print Value: 10804013 Numbering: – Type: volume Value: 1 – Type: issue Value: 3 Titles: – TitleFull: Mental Retardation & Developmental Disabilities Research Reviews Type: main |
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