Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin

…, N Sgarioto, MJ Hayes, LEL Romano… - Human molecular …, 2017 - academic.oup.com
Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is caused by
mutations in the gene SACS, encoding the 520 kDa protein sacsin. Although sacsin’s …

A reduction in Drp1-mediated fission compromises mitochondrial health in autosomal recessive spastic ataxia of Charlevoix Saguenay

TY Bradshaw, LEL Romano, EJ Duncan… - Human molecular …, 2016 - academic.oup.com
The neurodegenerative disease autosomal recessive spastic ataxia of Charlevoix Saguenay
(ARSACS) is caused by loss of function of sacsin, a modular protein that is required for …

[PDF][PDF] Multi-omic profiling reveals the ataxia protein sacsin is required for integrin trafficking and synaptic organization

LEL Romano, WY Aw, KM Hixson, TV Novoselova… - Cell Reports, 2022 - cell.com
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a childhood-onset
cerebellar ataxia caused by mutations in SACS, which encodes the protein sacsin. Cellular …

[HTML][HTML] GATA3 induces mitochondrial biogenesis in primary human CD4+ T cells during DNA damage

…, EC Carroll, C Garrod-Ketchley, LEL Romano… - Nature …, 2021 - nature.com
GATA3 is as a lineage-specific transcription factor that drives the differentiation of CD4 + T
helper 2 (Th2) cells, but is also involved in a variety of processes such as immune regulation, …

CCG• CGG interruptions in high‐penetrance SCA8 families increase RAN translation and protein toxicity

…, M Banez‐Coronel, T Zu, LEL Romano… - EMBO Molecular …, 2021 - embopress.org
Spinocerebellar ataxia type 8 (SCA8), a dominantly inherited neurodegenerative disorder
caused by a CTG•CAG expansion, is unusual because most individuals that carry the mutation …

[HTML][HTML] Oncometabolite induced primary cilia loss in pheochromocytoma

…, TV Novoselova, LEL Romano… - Endocrine-related …, 2019 - erc.bioscientifica.com
Primary cilia are sensory organelles involved in regulation of cellular signaling. Cilia loss is
frequently observed in tumors; yet, the responsible mechanisms and consequences for …

[HTML][HTML] AlphaFold predicted structure of the Hsp90-like domains of the neurodegeneration linked protein sacsin reveals key residues for ATPase activity

…, M Castelli, E Frasnetti, LEL Romano… - Frontiers in Molecular …, 2023 - frontiersin.org
The ataxia-linked protein sacsin has three regions of partial homology to Hsp90’s N-terminal
ATP binding domain. Although a crystal structure for this Hsp90-like domain has been …

The ataxia protein sacsin is required for integrin trafficking and synaptic organization

LEL Romano, WY Aw, KM Hixson, TV Novoselova… - bioRxiv, 2021 - biorxiv.org
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by
mutations in SACS, which manifest as a childhood-onset cerebellar ataxia. Cellular ARSACS …

GATA3 controls mitochondrial biogenesis in primary human CD4+ T cells during DNA damage

LA Callender, J Schroth, EC Carroll, LEL Romano… - bioRxiv, 2019 - biorxiv.org
Introductory paragraph GATA binding protein 3 (GATA3) has traditionally been regarded as
a lineage-specific transcription factor that drives the differentiation of CD4 + T helper (Th) 2 …

Search for a stochastic gravitational-wave signal in the second round of the Mock LISA Data Challenges

EL Robinson, JD Romano… - Classical and Quantum …, 2008 - iopscience.iop.org
… Antenna (LISA) relies on the combined use of two LISA channels, … of the second round of
the Mock LISA Data Challenges. For the … for more realistic studies of LISA’s performance and …