Article

Mutations in Hsp40 co-chaperone change the unique canonical inter-domain interactions stimulating LGMDD1 myopathy

2025-05-03

Abstract excerpt

Limb-girdle muscular dystrophy D1 (LGMDD1) is a rare, dominantly inherited neuromuscular disorder caused by mutations in the HSP40 co-chaperone DNAJB6, primarily in the GF or J-domains. Currently, no treatments are available, and a challenge in understanding the disease is identifying a specific client protein for DNAJB6 in skeletal muscle. Our previous research indicated that LGMDD1 GF domain mutants in Sis1 exhi...

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Literature Corpus work: 5facac93-ba2d-50b5-8321-d5aa5c3c2326
DOI: 10.1101/2025.05.02.651952

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