Protective Effects of 4-Phenylbutyrate Derivatives on the Neuronal Cell Death and Endoplasmic Reticulum Stress
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- Material Type
- 記事
- Author/Editor
- Seisuke MimoriYasunobu OkumaMasayuki Kaneko 他
- Author Heading
- Periodical title
- Biological and pharmaceutical bulletin
- No. or year of volume/issue
- 35(1):2012.1
- Volume
- 35
- Issue
- 1
- Pages
- 84-90
- Publication date of volume/issue (W3CDTF)
- 2012-01
- ISSN (Periodical Title)
- 0918-6158
- ISSN-L (Periodical Title)
- 0918-6158
- Publication (Periodical Title)
- Tokyo : Pharmaceutical Society of Japan
- Place of Publication (Country Code)
- JP
- Text Language Code
- eng
- NDLC
- Target Audience
- 一般
- Holding library
- 国立国会図書館
- Call No.
- Z53-V41
- Data Provider (Database)
- 国立国会図書館 : 国立国会図書館雑誌記事索引
- Bibliographic ID (NDL)
- 024029733
- Bibliographic Record Category (NDL)
- 632
- Summary, etc.
- Endoplasmic reticulum (ER) stress responses play an important role in neurodegenerative diseases. Sodium 4-phenylbutyrate (4-PBA) is a terminal aromatic substituted fatty acid that has been used for the treatment of urea cycle disorders. 4-PBA possesses <i>in vitro</i> chemical chaperone activity and reduces the accumulation of Parkin-associated endothelin receptor-like receptor (Pael-R), which is involved in autosomal recessive juvenile parkinsonism (AR-JP). In this study, we show that terminal aromatic substituted fatty acids, including 3-phenylpropionate (3-PPA), 4-PBA, 5-phenylvaleric acid, and 6-phenylhexanoic acid, prevented the aggregation of lactalbumin and bovine serum albumin. Aggregation inhibition increased relative to the number of carbons in the fatty acids. Moreover, these compounds protected cells against ER stress-induced neuronal cell death. The cytoprotective effect correlated with the <i>in vitro</i> chemical chaperone activity. Similarly, cell viability decreased on treatment with tunicamycin, an ER stress inducer, and was dependent on the number of carbons in the fatty acids. Moreover, the expression of glucose-regulated proteins 94 and 78 (GRP94, 78) decreased according to the number of carbons in the fatty acids. Furthermore, we investigated the effects of these compounds on the accumulation of Pael-R in neuroblastoma cells. 3-PPA and 4-PBA significantly suppressed neuronal cell death caused by ER stress induced by the overexpression of Pael-R. Overexpressed Pael-R accumulated in the ER of cells. With 3-PPA and 4-PBA treatment, the localization of the overexpressed Pael-R shifted away from the ER to the cytoplasmic membrane. These results suggest that terminal aromatic substituted fatty acids are potential candidates for the treatment of neurodegenerative diseases.
- DOI
- 10.1248/bpb.35.84
- Access Restrictions
- インターネット公開
- Data Provider (Database)
- 科学技術振興機構 : J-STAGE
- Summary, etc.
- Endoplasmic reticulum (ER) stress responses play an important role in neurodegenerative diseases. Sodium 4-phenylbutyrate (4-PBA) is a terminal aromatic substituted fatty acid that has been used for the treatment of urea cycle disorders. 4-PBA possesses <i>in vitro</i> chemical chaperone activity and reduces the accumulation of Parkin-associated endothelin receptor-like receptor (Pael-R), which is involved in autosomal recessive juvenile parkinsonism (AR-JP). In this study, we show that terminal aromatic substituted fatty acids, including 3-phenylpropionate (3-PPA), 4-PBA, 5-phenylvaleric acid, and 6-phenylhexanoic acid, prevented the aggregation of lactalbumin and bovine serum albumin. Aggregation inhibition increased relative to the number of carbons in the fatty acids. Moreover, these compounds protected cells against ER stress-induced neuronal cell death. The cytoprotective effect correlated with the <i>in vitro</i> chemical chaperone activity. Similarly, cell viability decreased on treatment with tunicamycin, an ER stress inducer, and was dependent on the number of carbons in the fatty acids. Moreover, the expression of glucose-regulated proteins 94 and 78 (GRP94, 78) decreased according to the number of carbons in the fatty acids. Furthermore, we investigated the effects of these compounds on the accumulation of Pael-R in neuroblastoma cells. 3-PPA and 4-PBA significantly suppressed neuronal cell death caused by ER stress induced by the overexpression of Pael-R. Overexpressed Pael-R accumulated in the ER of cells. With 3-PPA and 4-PBA treatment, the localization of the overexpressed Pael-R shifted away from the ER to the cytoplasmic membrane. These results suggest that terminal aromatic substituted fatty acids are potential candidates for the treatment of neurodegenerative diseases.
- DOI
- 10.1248/bpb.35.84
- Access Restrictions
- インターネット公開
- Related Material (URI)
- Is Referenced By
- Evaluation of synthetic naphthalene derivatives as novel chemical chaperones that mimic 4-phenylbutyric acidSpecies Differences in the Binding of Sodium 4-Phenylbutyrate to Serum AlbuminNeuroprotection by Endoplasmic Reticulum Stress-Induced HRD1 and Chaperones: Possible Therapeutic Targets for Alzheimer’s and Parkinson’s DiseaseIndole-3-propionic acid has chemical chaperone activity and suppresses endoplasmic reticulum stress-induced neuronal cell death4-Phenylbutyric acid protects against neuronal cell death by primarily acting as a chemical chaperone rather than histone deacetylase inhibitorNeuroprotective effect of S-allyl-l-cysteine derivatives against endoplasmic reticulum stress-induced cytotoxicity is independent of calpain inhibitionPossible involvement of endoplasmic reticulum stress in the pathogenesis of Alzheimer’s diseaseIdentification of Novel Oxindole Compounds That Suppress ER Stress-Induced Cell Death as Chemical ChaperonesSKF-10047, a prototype Sigma-1 receptor agonist, augmented the membrane trafficking and uptake activity of the serotonin transporter and its C-terminus-deleted mutant via a Sigma-1 receptor-independent mechanismEndoplasmic Reticulum Stress and Parkinson’s Disease: The Role of HRD1 in Averting Apoptosis in Neurodegenerative DiseaseA unique compound ameliorating endoplasmic reticulum stress and insulin resistance by binding to β tubulin神経変性疾患における小胞体折りたたみ不全タンパク質処理応答に関する薬理学的研究小胞体ストレス応答機構の分子薬理学的研究：神経変性疾患の予防・治療をめざしてDiscovery of synthetic methoxysubstituted - phenylbutyric acid derivatives as chemical chaperons
- References
- The Therapeutic Potential of HDAC Inhibitors in the Treatment of Multiple SclerosisThe molten globule state as a clue for understanding the folding and cooperativity of globular‐protein structurePotent inhibition of scrapie prion replication in cultured cells by bis-acridinesIn vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.Neurodegeneration of mouse nigrostriatal dopaminergic system induced by repeated oral administration of rotenone is prevented by 4‐phenylbutyrate, a chemical chaperoneMutations in the parkin gene cause autosomal recessive juvenile parkinsonismTranslational control by the ER transmembrane kinase/ribonuclease IRE1 under ER stressOnly the Reduced Conformer of -Lactalbumin Is Inducible to Aggregation by Protein AggregatesCloning of mammalian Ire1 reveals diversity in the ER stress responsesA chemical chaperone, sodium 4-phenylbutyric acid, attenuates the pathogenic potency in human α-synuclein A30P + A53T transgenic miceParkin Suppresses Unfolded Protein Stress-induced Cell Death through Its E3 Ubiquitin-protein Ligase ActivityHistone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathway4‐Phenylbutyrate restores the functionality of a misfolded mutant low‐density lipoprotein receptorPilot trial of phenylbutyrate in spinal muscular atrophyParkin Suppresses Dopaminergic Neuron-Selective Neurotoxicity Induced by Pael-R in DrosophilaSodium 4-Phenylbutyrate Protects against Cerebral Ischemic InjuryTargeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosisNeuroprotective Effects of Phenylbutyrate in the N171-82Q Transgenic Mouse Model of Huntington's DiseasePivotal role of oligomerization in expanded polyglutamine neurodegenerative disordersChemical Chaperones Reduce ER Stress and Restore Glucose Homeostasis in a Mouse Model of Type 2 DiabetesA single amino acid substitution differentiates Hsp70-dependent effects on α-synuclein degradation and toxicityPhenylbutyrate Ameliorates Cognitive Deficit and Reduces Tau Pathology in an Alzheimer's Disease Mouse ModelAn Unfolded Putative Transmembrane Polypeptide, which Can Lead to Endoplasmic Reticulum Stress, Is a Substrate of ParkinPrevention of Transthyretin Amyloid Disease by Changing Protein Misfolding EnergeticsSuppressive effects of 4‐phenylbutyrate on the aggregation of Pael receptors and endoplasmic reticulum stress
- Data Provider (Database)
- 国立情報学研究所 : CiNii Research
- Original Data Provider (Database)
- Japan Link Center学術機関リポジトリデータベース雑誌記事索引データベースCrossrefPubMedCiNii Articles科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベース科学研究費助成事業データベースCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossrefCrossref
- Bibliographic ID (NDL)
- 024029733
- NAID
- 130001872347