About: Altered lipid metabolism in a Drosophila model of Friedreich's ataxia

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About: Altered lipid metabolism in a Drosophila model of Friedreich's ataxia Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : covidontheweb.inria.fr associated with source document(s)

Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Altered lipid metabolism in a Drosophila model of Friedreich's ataxia
Creator	Botella, José Ganfornina, María Liebisch, Gerhard Moltó, María Navarro, Juan Ohmann, Elisabeth Sanchez, Diego Schmitz, Gerd Schneuwly, Stephan
Source	PMC
abstract	Friedreich's ataxia (FRDA) is the most common form of autosomal recessive ataxia caused by a deficit in the mitochondrial protein frataxin. Although demyelination is a common symptom in FRDA patients, no multicellular model has yet been developed to study the involvement of glial cells in FRDA. Using the recently established RNAi lines for targeted suppression of frataxin in Drosophila, we were able to study the effects of general versus glial-specific frataxin downregulation. In particular, we wanted to study the interplay between lowered frataxin content, lipid accumulation and peroxidation and the consequences of these effects on the sensitivity to oxidative stress and fly fitness. Interestingly, ubiquitous frataxin reduction leads to an increase in fatty acids catalyzing an enhancement of lipid peroxidation levels, elevating the intracellular toxic potential. Specific loss of frataxin in glial cells triggers a similar phenotype which can be visualized by accumulating lipid droplets in glial cells. This phenotype is associated with a reduced lifespan, an increased sensitivity to oxidative insult, neurodegenerative effects and a serious impairment of locomotor activity. These symptoms fit very well with our observation of an increase in intracellular toxicity by lipid peroxides. Interestingly, co-expression of a Drosophila apolipoprotein D ortholog (glial lazarillo) has a strong protective effect in our frataxin models, mainly by controlling the level of lipid peroxidation. Our results clearly support a strong involvement of glial cells and lipid peroxidation in the generation of FRDA-like symptoms.
has issue date	2010-07-15(xsd:dateTime)
bibo:doi	10.1093/hmg/ddq183
bibo:pmid	20460268
has license	no-cc
sha1sum (hex)	966d4620700365cee6c1e5731ff4e8e47a56af9d
schema:url	https://doi.org/10.1093/hmg/ddq183
resource representing a document's title	Altered lipid metabolism in a Drosophila model of Friedreich's ataxia
has PubMed Central identifier	PMC7108586
has PubMed identifier	20460268
schema:publication	Hum Mol Genet
resource representing a document's body	covid:966d4620700365cee6c1e5731ff4e8e47a56af9d#body_text
is schema:about of	named entity 'ataxia' named entity 'controlling' named entity 'content' named entity 'model' named entity 'Drosophila' named entity 'Our' named entity 'levels' named entity 'Friedreich' named entity '27S' named entity 'VERY WELL' named entity 'OBSERVATION' named entity 'LINES' named entity 'LIPID DROPLETS' named entity 'INSULT' named entity 'RNAI' named entity 'MODELS' named entity 'INVOLVEMENT' named entity 'REDUCED LIFESPAN' named entity 'PHENOTYPE' named entity 'LEADS' named entity 'MULTICELLULAR' named entity 'DOWNREGULATION' named entity 'SYMPTOM' named entity 'PATIENTS' named entity 'SPECIFIC' covid:arg/966d4620700365cee6c1e5731ff4e8e47a56af9d named entity 'INTRACELLULAR' named entity 'TOXIC POTENTIAL' named entity 'THESE' named entity 'FLY' named entity 'LOSS OF' named entity 'LIKE' named entity 'APOLIPOPROTEIN D' named entity 'IMPAIRMENT' named entity 'UBIQUITOUS' named entity 'ENHANCEMENT' named entity 'GLIAL CELLS' named entity 'OXIDATIVE STRESS' named entity 'CO-EXPRESSION' named entity 'TOXICITY' named entity 'WANTED' named entity 'RESULTS' named entity 'INCREASED SENSITIVITY' named entity 'ESTABLISHED' named entity 'LEVELS' named entity 'SYMPTOMS' named entity 'ASSOCIATED WITH' named entity 'SUPPORT' named entity 'OUR' named entity 'DROSOPHILA' named entity 'PROTECTIVE EFFECT' named entity 'CONTENT' named entity 'GENERATION' named entity 'COMMON' named entity 'PARTICULAR' named entity 'LIPID PEROXIDATION' named entity 'MITOCHONDRIAL PROTEIN' named entity 'LOWERED' named entity 'LEVEL' named entity 'FRATAXIN' named entity 'USING' named entity 'FRDA' named entity 'A COMMON' named entity 'AUTOSOMAL RECESSIVE' named entity 'FATTY ACIDS' named entity 'ATAXIA' named entity 'STRONG' named entity 'INCREASE' named entity 'CAUSED BY' named entity 'FORM' named entity 'ORTHOLOG' named entity 'REDUCTION' named entity 'DEMYELINATION'

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