| Literature DB >> 26906425 |
James B Summerville1, Joseph F Faust1, Ethan Fan1, Diana Pendin2, Andrea Daga3, Joseph Formella1, Michael Stern4, James A McNew4.
Abstract
Hereditary spastic paraplegia (HSP) is a set of genetic diseases caused by mutations in one of 72 genes that results in age-dependent corticospinal axon degeneration accompanied by spasticity and paralysis. Two genes implicated in HSPs encode proteins that regulate endoplasmic reticulum (ER) morphology. Atlastin 1 (ATL1, also known as SPG3A) encodes an ER membrane fusion GTPase and reticulon 2 (RTN2, also known as SPG12) helps shape ER tube formation. Here, we use a new fluorescent ER marker to show that the ER within wild-type Drosophila motor nerve terminals forms a network of tubules that is fragmented and made diffuse upon loss of the atlastin 1 ortholog atl. atl or Rtnl1 loss decreases evoked transmitter release and increases arborization. Similar to other HSP proteins, Atl inhibits bone morphogenetic protein (BMP) signaling, and loss of atl causes age-dependent locomotor deficits in adults. These results demonstrate a crucial role for ER in neuronal function, and identify mechanistic links between ER morphology, neuronal function, BMP signaling and adult behavior.Entities:
Keywords: Atlastin; ER; Neuron; Reticulon
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Year: 2016 PMID: 26906425 PMCID: PMC4852773 DOI: 10.1242/jcs.184929
Source DB: PubMed Journal: J Cell Sci ISSN: 0021-9533 Impact factor: 5.285