Abstract:
:Mutations in the nuclear matrix protein Matrin 3 (MATR3) have been identified in amyotrophic lateral sclerosis and myopathy. To investigate the mechanisms underlying MATR3 mutations in neuromuscular diseases and efficiently screen for modifiers of MATR3 toxicity, we generated transgenic MATR3 flies. Our findings indicate that expression of wild-type or mutant MATR3 in motor neurons reduces climbing ability and lifespan of flies, while their expression in indirect flight muscles (IFM) results in abnormal wing positioning and muscle degeneration. In both motor neurons and IFM, mutant MATR3 expression results in more severe phenotypes than wild-type MATR3, demonstrating that the disease-linked mutations confer pathogenicity. We conducted a targeted candidate screen for modifiers of the MATR3 abnormal wing phenotype and identified multiple enhancers involved in axonal transport. Knockdown of these genes enhanced protein levels and insolubility of mutant MATR3. These results suggest that accumulation of mutant MATR3 contributes to toxicity and implicate axonal transport dysfunction in disease pathogenesis.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Zhao M,Kao CS,Arndt C,Tran DD,Cho WI,Maksimovic K,Chen XXL,Khan M,Zhu H,Qiao J,Peng K,Hong J,Xu J,Kim D,Kim JR,Lee J,van Bruggen R,Yoon WH,Park Jdoi
10.1002/1873-3468.13858subject
Has Abstractpub_date
2020-09-01 00:00:00pages
2800-2818issue
17eissn
0014-5793issn
1873-3468journal_volume
594pub_type
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