1.
Adv Exp Med Biol
; 949: 227-243, 2016.
Article
in English
| MEDLINE
| ID: mdl-27714692
ABSTRACT
Astrocytes play crucial roles in maintaining brain homeostasis and in orchestrating neural development, all through tightly coordinated steps that cooperate to maintain the balance needed for normal development. Here, we review the alterations in astrocyte functions that contribute to a variety of developmental neurometabolic disorders and provide additional data on the predominant role of astrocyte dysfunction in the neurometabolic neurodegenerative disease glutaric acidemia type I. Finally, we describe some of the therapeutical approaches directed to neurometabolic diseases and discuss if astrocytes can be possible therapeutic targets for treating these disorders.
Subject(s)
Amino Acid Metabolism, Inborn Errors/diagnosis , Amino Acid Metabolism, Inborn Errors/therapy , Astrocytes/pathology , Brain Diseases, Metabolic/diagnosis , Brain Diseases, Metabolic/therapy , Brain/pathology , Glutaryl-CoA Dehydrogenase/deficiency , Alexander Disease/diagnosis , Alexander Disease/metabolism , Alexander Disease/pathology , Alexander Disease/therapy , Amino Acid Metabolism, Inborn Errors/metabolism , Amino Acid Metabolism, Inborn Errors/pathology , Antioxidants/therapeutic use , Astrocytes/drug effects , Astrocytes/metabolism , Brain/drug effects , Brain/metabolism , Brain Diseases, Metabolic/metabolism , Brain Diseases, Metabolic/pathology , Ceruloplasmin/deficiency , Ceruloplasmin/metabolism , Diet/methods , Disease Management , Glucose/therapeutic use , Glutamate-Ammonia Ligase/deficiency , Glutamate-Ammonia Ligase/metabolism , Glutaryl-CoA Dehydrogenase/metabolism , Hepatic Encephalopathy/diagnosis , Hepatic Encephalopathy/metabolism , Hepatic Encephalopathy/pathology , Hepatic Encephalopathy/therapy , Homeostasis , Humans , Iron Metabolism Disorders/diagnosis , Iron Metabolism Disorders/metabolism , Iron Metabolism Disorders/pathology , Iron Metabolism Disorders/therapy , Neurodegenerative Diseases/diagnosis , Neurodegenerative Diseases/metabolism , Neurodegenerative Diseases/pathology , Neurodegenerative Diseases/therapy , Neurogenesis/drug effects , Niemann-Pick Disease, Type C/diagnosis , Niemann-Pick Disease, Type C/metabolism , Niemann-Pick Disease, Type C/pathology , Niemann-Pick Disease, Type C/therapy , Pyruvate Carboxylase Deficiency Disease/diagnosis , Pyruvate Carboxylase Deficiency Disease/metabolism , Pyruvate Carboxylase Deficiency Disease/pathology , Pyruvate Carboxylase Deficiency Disease/therapy , Sorption Detoxification
2.
Nihon Rinsho
; 60 Suppl 4: 755-8, 2002 Apr.
Article
in Japanese
| MEDLINE
| ID: mdl-12013992
Subject(s)
Pyruvate Carboxylase Deficiency Disease , Acidosis, Lactic/etiology , Child, Preschool , Citrullinemia/etiology , Diagnosis, Differential , Humans , Hyperammonemia/etiology , Infant , Infant, Newborn , Mutation , Prognosis , Pyruvate Carboxylase/genetics , Pyruvate Carboxylase Deficiency Disease/diagnosis , Pyruvate Carboxylase Deficiency Disease/etiology , Pyruvate Carboxylase Deficiency Disease/therapy
3.
Clin Pediatr (Phila)
; 40(9): 519-21, 2001 Sep.
Article
in English
| MEDLINE
| ID: mdl-11583052