Journal Article

DZNE-2023-00258

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Spatial proteomics reveals secretory pathway disturbances caused by neuropathy-associated TECPR2.

Nalbach, K. ; Schifferer, M.DZNE* ; Bhattacharya, D. ; Ho-Xuan, H. ; Tseng, W. ; Williams, L. A. ; Stolz, A. ; Lichtenthaler, S. F.DZNE* ; Elazar, Z. ; Behrends, C.

2023
Nature Publishing Group UK [London]

This record in other databases:    

Please use a persistent id in citations: doi:10.1038/s41467-023-36553-6

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.

Keyword(s): Carrier Proteins: metabolism (MeSH) ; Secretory Pathway (MeSH) ; Proteomics (MeSH) ; Protein Transport (MeSH) ; Autophagosomes (MeSH) ; Autophagy: genetics (MeSH) ; Golgi Apparatus (MeSH) ; Nerve Tissue Proteins: metabolism (MeSH) ; Carrier Proteins ; Nerve Tissue Proteins

Note: CC BY

---

Contributing Institute(s):

1. Neuronal Cell Biology (AG Misgeld)
2. Neuroproteomics (AG Lichtenthaler)

Research Program(s):

1. 351 - Brain Function (POF4-351) (POF4-351)
2. 352 - Disease Mechanisms (POF4-352) (POF4-352)

Appears in the scientific report 2023

---

Database coverage:
; ; ; ; Article Processing Charges ; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Agriculture, Biology and Environmental Sciences ; Current Contents - Life Sciences ; Current Contents - Physical, Chemical and Earth Sciences ; DOAJ Seal ; Essential Science Indicators ; Fees ; IF >= 15 ; JCR ; PubMed Central ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection ; Zoological Record

---

---

Linked articles:

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Journal Article (Erratum/Correction) Nalbach, K.Extern* ; Schifferer, M.DZNE* ; Bhattacharya, D. ; Ho-Xuan, H. ; Tseng, W. C. ; Williams, L. A. ; Stolz, A. ; Lichtenthaler, S. F.DZNE* ; Elazar, Z. ; Behrends, C.
Author Correction: Spatial proteomics reveals secretory pathway disturbances caused by neuropathy-associated TECPR2.
Nature Communications 14(1), 8322 (2023) [10.1038/s41467-023-43990-w]2023   Files  Fulltext by Pubmed Central BibTeX | EndNote: XML, Text | RIS

---