Table 4.
Drosophila models of Huntington’s disease.
Drosophila Model | Developmental Stage | Assay Used for Neuropathology | References |
---|---|---|---|
Huntington’s Disease | |||
Drosophila orthologs of human genes | |||
Fly dHtt does not express polyQ in its N–terminus | N/A | N/A | [141] |
Overexpression of human transgenes | |||
Transgenic expression of a Q48 peptide or Htt Exon1p in neurons | Adult | Lifespan, photoreceptor morphology count | [144] |
Transgenic expression of various Q48 constructs | Adult | Locomotor, photoreceptor morphology count | [151] |
Transgenic expression of Q108 and Q48 peptides, transgenic expression of bivalent polyQ peptides | Adult | Lifespan, photoreceptor morphology count | [152] |
Expression of Q127 and Q20 peptides | Adult | SEM and light microscopy for retina morphology, light microscopy for pigmentation defects, staining with FITC for presence of polyQ aggregates | [153] |
Expression of mRFP–tagged N–terminal fragments of human Q15 or Q138 peptides | Adult | Lifespan, locomotion, activated–Caspase 3 immunostaining, immunostaining for brain size | [148] |
Expression of an mRFP–tagged N–terminal fragment of human Q15 or Q138 peptides containing exons 1–12 | Adult | Immunofluorescence for spreading of Huntingtin aggregates in the brain | [25] |
Expression of several 3– or 1–exon sections of mutant Htt with various polyQ lengths in clock neurons and ventral lateral neurons (sLNvs), RNAi knockdown of heat shock protein (Hsp) | Adult | Htt–eGFP fusions to track and quantify aggregation, sLNv count, rhythmicity, confocal imaging for PER protein intensity, transcript levels of Hop–associated proteins | [145] |
Expression of Q93 and Q20 peptides | Adult, Larva | Adult: locomotion Larva: crawling assay |
[146] |
Temperature–inducible expression of a Q15 or Q138 12–exon fragment of the human Htt gene, or expression of a 548 amino acid Q0 or Q128 segment of human Htt | Adult, pharate adult, larva |
Adult: RFP tag for imaging of Htt aggregation and localization Pharate adult: lethality Larvae: viability past 2nd instar for small molecule screen, Fluorescence recovery after photobleaching for aggregate growth |
[142] |
Expression of full–length Q128 or Q16 human Htt | Adult, larva | Adults: Western blot for Huntingtin levels, photoreceptor morphology count, locomotion, flying assay, confocal microscopy to count neuronal projections into IFMs Larvae: immunohistochemistry for third–instar larval NMJ count, EJP amplitudes, Ca2+ imaging |
[143] |