Don W. Cleveland

This paper develops a multimodal spatial transcriptomics approach to study CAG repeat expansion, huntingtin aggregation, and neuron loss in Huntington's Disease using human brain tissue.

MERFISHEYES provides a web-based platform for visualizing and sharing spatial transcriptomics data, enabling interactive exploration and cross-dataset comparisons.

This study shows that mutant SOD1 aggregates in myelinic nanochannels and accelerates ALS progression in mice, highlighting the role of myelin channel integrity in disease.

The paper shows statins and mevalonate pathway inhibition increase STMN2 and ATF3, promoting neuronal regeneration in neurodegenerative disease.

This study develops a gene therapy strategy using snRNAs to correct mis-splicing of STMN2 and UNC13A in TDP-43 proteinopathies, showing restoration of protein levels and axonal regeneration in motor neurons.

This study identifies acetylation of lysine 82 in TDP-43 as a mechanism causing nuclear loss of function and cytoplasmic accumulation, linked to neurodegenerative diseases like ALS.

This study shows that injecting human FUS fibrils into mice with human FUS causes FUS aggregation and FTLD-like disease progression, with prion-like spread and cognitive deficits.

This paper demonstrates that transient suppression of PTBP1 in aged glial cells can re-activate neurogenesis in murine and human brains, generating new neurons through canonical pathways.

This study identifies TDP-43 dysfunction and reduced m6A modification as mechanisms causing aberrant splicing in Huntington's disease using mouse and human brain data.

The paper identifies and characterizes a reference iPSC line, KOLF2.1J, for standardized use in collaborative studies across multiple differentiation protocols and assays.

This study evaluates human-derived anti-poly-GA antibodies in cellular and animal models of C9ORF72 disease, showing mixed effects on poly-GA levels and disease progression.

The paper identifies ELAVL3 as a downregulated RNA binding protein in ALS, showing nuclear depletion and mislocalization in motor neurons, with findings suggesting loss of function rather than cytoplasmic toxicity.

This study shows that wild-type FUS rescues ALS-like disease caused by mutant FUS by activating an autoregulatory loop that reduces cytoplasmic mutant FUS levels.

This study converts astrocytes into functional neurons to reverse Parkinson's disease in mice by suppressing PTB, restoring dopamine levels and nigrostriatal pathways.