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@ARTICLE{Nalbach:255139,
author = {Nalbach, Karsten and Schifferer, Martina and Bhattacharya,
Debjani and Ho-Xuan, Hung and Tseng, Wei and Williams, Luis
A and Stolz, Alexandra and Lichtenthaler, Stefan F and
Elazar, Zvulun and Behrends, Christian},
title = {{S}patial proteomics reveals secretory pathway disturbances
caused by neuropathy-associated {TECPR}2.},
journal = {Nature Communications},
volume = {14},
number = {1},
issn = {2041-1723},
address = {[London]},
publisher = {Nature Publishing Group UK},
reportid = {DZNE-2023-00258},
pages = {870},
year = {2023},
note = {CC BY},
abstract = {Hereditary sensory and autonomic neuropathy 9 (HSAN9) is a
rare fatal neurological disease caused by mis- and nonsense
mutations in the gene encoding for Tectonin β-propeller
repeat containing protein 2 (TECPR2). While TECPR2 is
required for lysosomal consumption of autophagosomes and
ER-to-Golgi transport, it remains elusive how exactly TECPR2
is involved in autophagy and secretion and what downstream
sequels arise from defective TECPR2 due to its involvement
in these processes. To address these questions, we determine
molecular consequences of TECPR2 deficiency along the
secretory pathway. By employing spatial proteomics, we
describe pronounced changes with numerous proteins important
for neuronal function being affected in their intracellular
transport. Moreover, we provide evidence that TECPR2's
interaction with the early secretory pathway is not
restricted to COPII carriers. Collectively, our systematic
profiling of a HSAN9 cell model points to specific
trafficking and sorting defects which might precede
autophagy dysfunction upon TECPR2 deficiency.},
keywords = {Carrier Proteins: metabolism / Secretory Pathway /
Proteomics / Protein Transport / Autophagosomes / Autophagy:
genetics / Golgi Apparatus / Nerve Tissue Proteins:
metabolism / Carrier Proteins (NLM Chemicals) / Nerve Tissue
Proteins (NLM Chemicals)},
cin = {AG Misgeld / AG Lichtenthaler},
ddc = {500},
cid = {I:(DE-2719)1110000-4 / I:(DE-2719)1110006},
pnm = {351 - Brain Function (POF4-351) / 352 - Disease Mechanisms
(POF4-352)},
pid = {G:(DE-HGF)POF4-351 / G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:36797266},
pmc = {pmc:PMC9935918},
doi = {10.1038/s41467-023-36553-6},
url = {https://pub.dzne.de/record/255139},
}