PXD016661

PXD016661 is an original dataset announced via ProteomeXchange.

Title	FAM134B oligomerization drives ER membrane scission for ER-phagy
Description	ER degradation by selective autophagy (ER-phagy) is crucial for ER homeostasis. However, it remains unclear how ER scission is regulated for subsequent autophagosomal sequestration and lysosomal degradation. Here, we show that oligomer formation of FAM134B (also referred to as reticulophagy regulator 1 or RETREG1) through its Reticulon domain was required for membrane fragmentation in vitro and ER-phagy in vivo. Under ER stress conditions, activated CAMK2B phosphorylated the Reticulon domain of FAM134B, which enhanced FAM134B oligomerization and activity in membrane fragmentation to accommodate high demand of ER-phagy. Unexpectedly, FAM134BG216R, a variant derived from a type II HSAN patient, exhibited gain-of-function defects, such as hyperactive self-association and membrane scission, which resulted in excessive ER-phagy and sensory neuron death. Therefore, this study revealed a mechanism of ER membrane fragmentation in ER-phagy, along with a signaling pathway in regulating ER turnover, and suggested a potential implication of excessive selective autophagy in human diseases.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:59:57.221.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Jingzi Zhang
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	phosphorylated residue
Instrument	LTQ Orbitrap Velos; TripleTOF 5600

Revision	Datetime	Status	ChangeLog Entry
0	2019-12-09 05:30:43	ID requested
1	2020-01-09 10:55:17	announced
2	2020-01-20 00:42:20	announced	2020-01-20: Updated publication reference for PubMed record(s): 31930741.
⏵ 3	2024-10-22 04:59:58	announced	2024-10-22: Updated project metadata.

10.15252/embj.2019102608;

Jiang X, Wang X, Ding X, Du M, Li B, Weng X, Zhang J, Li L, Tian R, Zhu Q, Chen S, Wang L, Liu W, Fang L, Neculai D, Sun Q, FAM134B oligomerization drives endoplasmic reticulum membrane scission for ER-phagy. EMBO J, 39(5):e102608(2020) [pubmed]

submitter keyword: ER-phagy

FAM134B oligomerization

membrane fragmentation

ER stress

CAMK2B

Qiming Sun
contact affiliation	Department of Biochemistry, and Department of Cardiology of Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310058, China.
contact email	qmsun@zju.edu.cn
lab head
Jingzi Zhang
contact affiliation	Nanjing University
contact email	zhangjz@nicemice.cn
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2020/01/PXD016661

PRIDE project URI

• PRIDE
  1. PXD016661
     1. Label: PRIDE project
     2. Name: FAM134B oligomerization drives ER membrane scission for ER-phagy