User profiles for J. N. Stankowski

Jeannette Stankowski

Instructor in Neuroscience
Verified email at mayo.edu
Cited by 2839

C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

…, PO Bauer, EC Whitelaw, L Rousseau, JN Stankowski… - Science, 2015 - science.org
The major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis is a G
4 C 2 repeat expansion in C9ORF72. Efforts to combat neurodegeneration associated with “…

[HTML][HTML] Aggregation-prone c9FTD/ALS poly (GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

…, J Chew, VV Belzil, M Prudencio, JN Stankowski… - Acta …, 2014 - Springer
The occurrence of repeat-associated non-ATG (RAN) translation, an atypical form of translation
of expanded repeats that results in the synthesis of homopolymeric expansion proteins, …

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

…, M Shinohara, WL Lin, A Garrett, JN Stankowski… - Nature …, 2016 - nature.com
Neuronal inclusions of poly(GA), a protein unconventionally translated from G 4 C 2 repeat
expansions in C9ORF72, are abundant in patients with frontotemporal dementia (FTD) and …

Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis

TF Gendron, J Chew, JN Stankowski… - Science translational …, 2017 - science.org
There is no effective treatment for amyotrophic lateral sclerosis (ALS), a devastating motor
neuron disease. However, discovery of a G 4 C 2 repeat expansion in the C9ORF72 gene as …

(Patho‐) physiological relevance of PINK 1‐dependent ubiquitin phosphorylation

…, EL Moussaud‐Lamodière, JN Stankowski… - EMBO …, 2015 - embopress.org
Mutations in PINK 1 and PARKIN cause recessive, early‐onset Parkinson's disease ( PD ).
Together, these two proteins orchestrate a protective mitophagic response that ensures the …

[HTML][HTML] Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

…, M Castanedes-Casey, A Kurti, JN Stankowski… - Molecular …, 2019 - Springer
Background AG 4 C 2 hexanucleotide repeat expansion in the noncoding region of C9orf72
is the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (…

[HTML][HTML] Acetylation: a new key to unlock tau's role in neurodegeneration

C Cook, JN Stankowski, Y Carlomagno… - Alzheimer's research & …, 2014 - Springer
The identification of tau protein as a major constituent of neurofibrillary tangles spurred
considerable effort devoted to identifying and validating pathways through which therapeutics …

Manganese exposure is cytotoxic and alters dopaminergic and GABAergic neurons within the basal ganglia

…, DJ Anderson, JN Stankowski… - Journal of …, 2009 - Wiley Online Library
Manganese is an essential nutrient, integral to proper metabolism of amino acids, proteins
and lipids. Excessive environmental exposure to manganese can produce extrapyramidal …

[HTML][HTML] The lysosomal protein cathepsin L is a progranulin protease

CW Lee, JN Stankowski, J Chew, CN Cook… - Molecular …, 2017 - Springer
Haploinsufficiency of GRN, the gene encoding progranulin (PGRN), causes frontotemporal
lobar degeneration (FTLD), the second most common cause of early-onset dementia. …

Therapeutic targets for neuroprotection in acute ischemic stroke: lost in translation?

JN Stankowski, R Gupta - Antioxidants & redox signaling, 2011 - liebertpub.com
The development of a suitable neuroprotective agent to treat ischemic stroke has failed when
transitioned to the clinical setting. An understanding of the molecular mechanisms involved …