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Distinct amyloid fibril structures formed by ALS-causing SOD1 mutants G93A and D101N

ABSTRACT: We expressed and purified recombinant full-length wild-type human SOD1 and its G93A and D101N variants in Expi293F cells where the initiating Met was removed and the N-terminus at Ala1 was acetylated, and identified N-terminal acetylation of the mammalian cell–purified G93A , D101N , and wild-type SOD1 using mass spectrometry (MS). Analysis of the b-ions inindicated +42.01037, +42.01014, and +42.01014 Da mass shifts representing the addition of an acetyl group to Ala1, demonstrating N-terminal acetylation in the mammalian cell–purified G93A, D101N, and wild-type SOD1.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Liqiang Wang

LAB HEAD: Yi Liang

PROVIDER: PXD066116 | Pride | 2025-10-20

REPOSITORIES: Pride

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			Action	DRS
	MS250427-D101N.msf	Msf
	MS250427-D101N.raw	Raw
	MS250427-G93A.msf	Msf
	MS250427-G93A.raw	Raw
	MS250427-WT.msf	Msf

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Publications

Distinct amyloid fibril structures formed by ALS-causing SOD1 mutants G93A and D101N.

Zhang Mu-Ya MY Ma Yeyang Y Wang Li-Qiang LQ Xia Wencheng W Li Xiang-Ning XN Zhao Kun K Chen Jie J Li Dan D Zou Liangyu L Wang Zhengzhi Z Liu Cong C Liang Yi Y

EMBO reports 20250826 19

Two hundred eight genetic mutations in SOD1 have been linked to amyotrophic lateral sclerosis (ALS). Of these, the G93A and D101N variants maintain much of their physiological function, closely resembling that of wild-type SOD1, and the SOD1-G93A transgenic mouse is the most extensively used mouse line in the study of ALS. In this study, we report two cryo-EM structures of amyloid fibrils formed by G93A and D101N mutants of SOD1 protein. These mutations give rise to amyloid fibrils with distinct ...[more]

PMID: 40859014

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