Ontology highlight
ABSTRACT:
INSTRUMENT(S): Orbitrap Exploris 480
ORGANISM(S): Homo Sapiens (human)
TISSUE(S): Cornea
SUBMITTER: Kimberly Jia Yi Low
LAB HEAD: Konstantin Pervushin
PROVIDER: PXD038281 | Pride | 2023-05-10
REPOSITORIES: Pride
Low Jia Yi Kimberly JYK Shi Xiangyan X Anandalakshmi Venkatraman V Neo Dawn D Peh Gary Swee Lim GSL Koh Siew Kwan SK Zhou Lei L Abdul Rahim M K MK Boo Ketti K Lee JiaXuan J Mohanram Harini H Alag Reema R Mu Yuguang Y Mehta Jodhbir S JS Pervushin Konstantin K
Communications biology 20230330 1
TGFBI-related corneal dystrophy (CD) is characterized by the accumulation of insoluble protein deposits in the corneal tissues, eventually leading to progressive corneal opacity. Here we show that ATP-independent amyloid-β chaperone L-PGDS can effectively disaggregate corneal amyloids in surgically excised human cornea of TGFBI-CD patients and release trapped amyloid hallmark proteins. Since the mechanism of amyloid disassembly by ATP-independent chaperones is unknown, we reconstructed atomic mo ...[more]