A model for PKC involvement in the pathogenesis of inborn errors of metabolism.
Trends Mol Med
; 8(11): 524-31, 2002 Nov.
Article
em En
| MEDLINE
| ID: mdl-12421686
ABSTRACT
A model for the possible involvement of Protein Kinase C (PKC) in the pathogenesis of inborn errors of metabolism has been proposed. According to this model, perturbation of PKC activity by the accumulation of naturally occurring compounds serves as a unifying functional link between genotype and phenotype. Recent reports regarding an increasing number of modulating metabolites, specific PKC-subtypes activities, their effect on transcription factors and gene expression in various diseases and additional PKC-substrates expand the model. A re-examination of the proposed model in view of these reports and, vice versa, a review of these reports in the context of the proposed model reveal some common phenotypic outcomes in inborn errors of fatty acid-, cholesterol- and homocystine-metabolism as well as lysosomal and peroxisomal diseases.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Proteína Quinase C
/
Esfingolipidoses
Tipo de estudo:
Etiology_studies
/
Prognostic_studies
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Trends Mol Med
Assunto da revista:
BIOLOGIA MOLECULAR
Ano de publicação:
2002
Tipo de documento:
Article
País de afiliação:
Austrália