Dataset Information

Proteomics analysis of the corneal amyloid disaggregation effects by brain chaperone L-PGDS

ABSTRACT: TGFBIp-related corneal dystrophy (CD) is the most common form of stromal corneal dystrophy. Although CD is a group of inheritable and progressive corneal diseases, non-inheritable cases of corneal amyloid deposition are reported. TGFBIp-CD is characterized by the accumulation of insoluble protein deposits consisting smaller proteolytic fragments of TGFBIp in the corneal tissues, eventually leading to progressive corneal opacity. Maximum density of these aggregates is in the corneal center implicating the local absence of amyloid controlling factors. Currently, no other treatment options are available besides the surgical replacement of the affected corneal tissues with a donor’s cornea. Here we show that neuroprotective ATP-independent amyloid β chaperone L-PGDS abundant in cerebrospinal fluid but absent in the corneal tissues can effectively disaggregate amyloids in vitro and in the surgically excised human cornea of patients with TGFBIp-CD.

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

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Dataset's files

Source:

			Action	DRS
	12Sep22_NTU_KIM_CTRL.raw	Raw
	12Sep22_NTU_KIM_LPGDS.raw	Raw
	12Sep22_NTU_Kimberly.sne	Other

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Publications

Release of frustration drives corneal amyloid disaggregation by brain chaperone.

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]

PMID: 36997596

Dataset Information

Proteomics analysis of the corneal amyloid disaggregation effects by brain chaperone L-PGDS

Dataset's files

Publications

Release of frustration drives corneal amyloid disaggregation by brain chaperone.

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