ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair

…, KK Gamage, R Kirchner, R Moccia… - Nature …, 2019 - nature.com
The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display
pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its gene …

A defined glycosaminoglycan-binding substratum for human pluripotent stem cells

JR Klim, L Li, PJ Wrighton, MS Piekarczyk… - Nature …, 2010 - nature.com
To exploit the full potential of human pluripotent stem cells for regenerative medicine,
developmental biology and drug discovery, defined culture conditions are needed. Media of known …

Modeling ALS with motor neurons derived from human induced pluripotent stem cells

…, LI Bruijn, S Chandran, K Eggan, R Ho, JR Klim… - Nature …, 2016 - nature.com
Directing the differentiation of induced pluripotent stem cells into motor neurons has allowed
investigators to develop new models of amyotrophic lateral sclerosis (ALS). However, …

[PDF][PDF] Genetic ablation of AXL does not protect human neural progenitor cells and cerebral organoids from Zika virus infection

…, RJ Ihry, S Kommineni, B Bilican, JR Klim… - Cell stem cell, 2016 - cell.com
Zika virus (ZIKV) can cross the placental barrier, resulting in infection of the fetal brain and
neurological defects including microcephaly. The cellular tropism of ZIKV and the identity of …

How to make spinal motor neurons

BN Davis-Dusenbery, LA Williams, JR Klim… - …, 2014 - journals.biologists.com
All muscle movements, including breathing, walking, and fine motor skills rely on the function
of the spinal motor neuron to transmit signals from the brain to individual muscle groups. …

[PDF][PDF] Loss of mouse Stmn2 function causes motor neuropathy

…, F Limone, AB Dorr, A Couto, G Pintacuda, BJ Joseph… - Neuron, 2022 - cell.com
Amyotrophic lateral sclerosis (ALS) is characterized by motor neuron degeneration accompanied
by aberrant accumulation and loss of function of the RNA-binding protein TDP43. Thus …

High-throughput discovery of synthetic surfaces that support proliferation of pluripotent cells

R Derda, S Musah, BP Orner, JR Klim, L Li… - Journal of the …, 2010 - ACS Publications
Synthetic materials that promote the growth or differentiation of cells have advanced the fields
of tissue engineering and regenerative medicine. Most functional biomaterials are based …

[PDF][PDF] Reactive astrocytes promote ALS-like degeneration and intracellular protein aggregation in human motor neurons by disrupting autophagy through TGF-β1

P Tripathi, N Rodriguez-Muela, JR Klim, AS de Boer… - Stem cell reports, 2017 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease.
Astrocytic factors are known to contribute to motor neuron degeneration and death in ALS. …

Exome sequencing in amyotrophic lateral sclerosis implicates a novel gene, DNAJC7, encoding a heat-shock protein

…, R Rademakers, JL McCauley, R Schüle… - Nature …, 2019 - nature.com
To discover novel genes underlying amyotrophic lateral sclerosis (ALS), we aggregated
exomes from 3,864 cases and 7,839 ancestry-matched controls. We observed a significant …

Connecting TDP-43 pathology with neuropathy

JR Klim, G Pintacuda, LA Nash, IG San Juan… - Trends in …, 2021 - cell.com
Transactive response DNA-binding protein 43 kDa (TDP-43), a multifunctional nucleic acid-binding
protein, is a primary component of insoluble aggregates associated with several …