Proteomics

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ARL6IP1 is an interacting partner of the ER-phagy receptor FAM134B

ABSTRACT: ER-resident membrane-shaping proteins with central reticulon homology domains (RHDs) have been associated with neurodegenerative disorders such as ARL6IP1 and FAM134B.Mutations in ARL6IP1 cause SPG61, a neurodegenerative disorder characterized by progressive leg spasticity (hereditary spastic paraplegia/HSP) in combination with loss of sensory and pain perception, thus reproducing typical symptoms of HSAN {Kurth, 2009 #8;Novarino, 2014 #28;Nizon, 2018 #162}.Our objetive was to analyse FAM134B-ARL6IP1 protein protein interactionS to underlying mechanisms, required for effective ER-remodelling and ER-phagy.

INSTRUMENT(S): Q Exactive HF

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Epithelial Cell

SUBMITTER: Adriana Covarrubias-Pinto  

LAB HEAD: Ivan Dikic

PROVIDER: PXD032718 | Pride | 2023-06-19

REPOSITORIES: Pride

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Membrane-shaping proteins characterized by reticulon homology domains play an important part in the dynamic remodelling of the endoplasmic reticulum (ER). An example of such a protein is FAM134B, which can bind LC3 proteins and mediate the degradation of ER sheets through selective autophagy (ER-phagy)<sup>1</sup>. Mutations in FAM134B result in a neurodegenerative disorder in humans that mainly affects sensory and autonomic neurons<sup>2</sup>. Here we report that ARL6IP1, another ER-shaping pr  ...[more]

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