Table 1.

Comparison of physiological hallmarks of FRDA in patients and phenotypes observed in FRDA fly models.

Patient Phenotype	Tissue Affected	Genotype	Fly Phenotype
Reduced longevity	Ubiquitous downregulation Ubiquitous downregulation Panneuronal Panneuronal	Actin-GAL4>UAS-fhIR Da-GAL4>UDIR Elav-GS>UDIR Elav-GAL4; UAS-Dicer2>UDIR	Shortened life span Developmental lethality Shortened life span Shortened life span
Ataxia	Ubiquitous downregulation Panneuronal Larval and adult CNS Serotonergic and Dopaminergic neurons	Actin-GAL4>UAS-fhIR Elav-GAL4; UAS-Dicer2>UDIR c698a-GAL4>UAS-fhIR Ddc-GAL4>UAS-fhIR	impaired locomotion impaired locomotion No effect No effect
Degeneration and atrophy of DN	mutant photoreceptor neurons	fh1	Degeneration of neuronal photoreceptors
Degeneration of large sensory neurons from the DRG	PNS PNS	C96-GAL4>UDIR Neur-GAL4>UAS-fhIR	Reduced longevity Reduced longevity, impaired locomotion
Degeneration of spinocerebellar tracts	Motorneurons	D42-GAL4>UDIR	No effect in life span, reduced mitochondrial transport and axonal degeneration
Demyelination of sural nerves and DRG fibres	Panglial	Repo-GAL4>UDIR	Shortened life span, impaired locomotion, brain degeneration, lipid dyshomeostasis
Hypertrophic cardiomyopathy	Heart	Hand-GS>UDIR	Heart dilatation an impairment of heart function
Abnormal muscle performance and recovery after exercise	Indirect Flight Muscles	Mef2-GAL4>UDIR	Reduced ATP production, shortened life span, impaired locomotion

CNS: Central Nervous System; DN: Dentatte nucleus; DRG: Dorsal root ganglia.