Amino acid replacement: W105term.
G24037238A
G?A
W104term | Miro-PC; W104term | Miro-PD; W104term | Miro-PE; W104term | Miro-PF
W105term
Reported as a W105 to UAG nonsense mutation.
bouton & neuromuscular junction | larval stage
bouton & neuromuscular junction | larval stage (with MiroSd32)
microtubule & bouton
mitochondrion & motor neuron | larval stage (with MiroSd32)
mitochondrion & neuron | larval stage
mitochondrion & neuron | larval stage (with MiroSd23)
mitochondrion & neuron | larval stage (with MiroSd26)
mitochondrion & neuron | larval stage (with MiroSd32)
mitochondrion & somatic muscle | larval stage
MiroB682/+ significantly reduces the frequency of mitochondrial transport events in axons of the wing marginal nerve, but does not significantly change the total number of mitochondria, as compared to controls.
In MiroB682 mutants the majority of mitochondria remain stranded in the neuronal soma and soma-proximal axon, whereas the distal axon and presynaptic nerve terminal are largely devoid of mitochondria. There is no evidence of NMJ degeneration or altered neuromuscular growth.
Stimulus-dependent or resting cytosolic Ca[2+] levels are not affected by the chronic lack of mitochondria in motor neuron-terminals of MiroB682 mutants.
Mutant neuromuscular junctions show reliable homeostatic compensation (increase in quantal content) after treatment with philanthotoxin-433 for 10 minutes.
In the motor neurons of MiroB682/MiroSd32 larvae, mitochondria are largely absent from neuro-muscular junctions, and are present in reduced numbers in axons, mainly concentrated close to the ventral ganglion. The proportion of motile mitochondria is significantly reduced in these cells and, unlike in wild-type, many stationary mitochondria are not clustered. In addition, the motile mitochondria (both anterograde and retrograde) are significantly shorter than in wild-type. There is also a small but significant reduction in the proportion of mitochondria that are motile and in the rate of mitochondrial flux in the axons of in motor neurons of MiroB682/+ larvae.
Gross eye morphology is normal but phototaxis is defective in flies whose eyes are homozygous for MiroB682.
Homozygous MiroB682 larvae are small and have reduced muscles. They tend to wiggle on the spot, rather than moving forwards. Anterior segments show rhythmic contractions, but posterior segments are sluggish, sometimes paralyzed.
In neurons of MiroB682/MiroB682, MiroSd23/MiroB682, MiroSd26/MiroB682 or MiroSd32/MiroB682 larvae, mitochondria are retained in the cell bodies and not properly distributed to neuronal processes - most neuromuscular junctions lack mitochondria. The somatic muscles of MiroB682/MiroB682 larvae also show highly abnormal distribution of mitochondria. However, the mitochondria in these animals show no structural abnormalities and have normal membrane potentials. In contrast to the effect on mitochondria, vesicle transport in the neurons of these animals is only weakly abnormal.
Bouton morphology and distribution is abnormal at neuromuscular junction in MiroB682/MiroB682 and MiroB682/MiroSd32 larvae. The arrangement of microtubules in the boutons of MiroB682/MiroB682 larvae is also abnormal, but the actin cytoskeleton in these boutons appears normal.
In MiroB682/MiroB682 larvae, resting Ca2+ concentration at neuro-muscular junctions is higher than normal.
In MiroSd32/MiroB682 larvae, excitatory junction potential evoked by 10Hz stimulation of neuro-muscular junctions (NMJs) starts off normal, but, unlike wild-type, rapidly decays. This phenotype is also seen in MiroB682/MiroB682, MiroB682/Df(3R)mbc-R1 or MiroB682/MiroSd26 larvae, but not in MiroB682/MiroSd23 larvae. Prolonged 10Hz stimulation also leads to greater increases in Ca2+ concentration at NMJs than in wild-type. MiroB682/MiroB682 also show a significant increase in spontaneous mini excitatory junction potentials following 10Hz stimulation of neuro-muscular junctions.
MiroB682 is a suppressor of abnormal neuroanatomy phenotype of α-SpecKK101541, Scer\GAL4elav-C155
MiroB682 has mitochondrion phenotype, non-enhanceable by Scer\GAL4elav-C155/α-SpecKK101541
MiroB682 is a suppressor of embryonic/larval neuromuscular junction phenotype of α-SpecKK101541, Scer\GAL4elav-C155
Homozygous MiroB682 suppresses the neuromuscular degeneration seen when α-SpecKK101541 is expressed under the control of Scer\GAL4elav-C155. The decrease in the number of mitochondria seen in MiroB682 mutants is also seen in these larvae.
MiroSd32/MiroB682 is partially rescued by Scer\GAL4RapGAP1-OK6/MiroUAS.Tag:MYC
MiroB682 is partially rescued by Scer\GAL4elav-C155/MiroUAS.cGa
MiroB682 is not rescued by Scer\GAL4how-24B/MiroUAS.cGa
MiroScer\UAS.cGa; Scer\GAL4elav-C155 rescues most of the phenotypes due to MiroB682/MiroB682 : larval lethality, larval body and muscle size, distribution of mitochondria to the distal extremities of neurons, neuro-muscular junction morphology and microtubule cytoskeleton organization. None of these phenotypes are rescued by MiroScer\UAS.cGa; Scer\GAL4how-24B.