[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis

J Root, P Merino, A Nuckols, M Johnson… - Neurobiology of disease, 2021 - Elsevier
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …

[HTML][HTML] Lysosomal cathepsins and their regulation in aging and neurodegeneration

V Stoka, V Turk, B Turk - Ageing research reviews, 2016 - Elsevier
Lysosomes and lysosomal hydrolases, including the cathepsins, have been shown to
change their properties with aging brain a long time ago, although their function was not …

[HTML][HTML] Genetics of the neuronal ceroid lipofuscinoses (Batten disease)

SE Mole, SL Cotman - Biochimica et Biophysica Acta (BBA)-Molecular …, 2015 - Elsevier
The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative
disorders that affect children and adults and are grouped together by similar clinical features …

[HTML][HTML] Cathepsins: Proteases that are vital for survival but can also be fatal

S Patel, A Homaei, HR El-Seedi, N Akhtar - Biomedicine & …, 2018 - Elsevier
The state of enzymes in the human body determines the normal physiology or pathology, so
all the six classes of enzymes are crucial. Proteases, the hydrolases, can be of several types …

Therapeutic landscape for Batten disease: current treatments and future prospects

TB Johnson, JT Cain, KA White… - Nature Reviews …, 2019 - nature.com
Batten disease (also known as neuronal ceroid lipofuscinoses) constitutes a family of
devastating lysosomal storage disorders that collectively represent the most common …

The future of cysteine cathepsins in disease management

L Kramer, D Turk, B Turk - Trends in pharmacological sciences, 2017 - cell.com
Since the discovery of the key role of cathepsin K in bone resorption, cysteine cathepsins
have been investigated by pharmaceutical companies as drug targets. The first clinical …

Recent updates on the genetics of amyotrophic lateral sclerosis and frontotemporal dementia

L Kirola, A Mukherjee, M Mutsuddi - Molecular Neurobiology, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) primarily affect the
motor and frontotemporal areas of the brain, respectively. These disorders share clinical …

Lysosomal storage diseases—the horizon expands

RMN Boustany - Nature Reviews Neurology, 2013 - nature.com
Since the discovery of the lysosome in 1955, advances have been made in understanding
the key roles and functions of this organelle. The concept of lysosomal storage diseases …

[HTML][HTML] NCL diseases—clinical perspectives

A Schulz, A Kohlschütter, J Mink, A Simonati… - … et Biophysica Acta (BBA …, 2013 - Elsevier
The neuronal ceroid lipofuscinoses (NCLs) are lysosomal storage disorders and together
are the most common degenerative brain diseases in childhood. They are a group of …

[HTML][HTML] Cysteine cathepsins: A long and winding road towards clinics

M Biasizzo, U Javoršek, E Vidak, M Zarić… - Molecular Aspects of …, 2022 - Elsevier
Biomedical research often focuses on properties that differentiate between diseased and
healthy tissue; one of the current focuses is elevated expression and altered localisation of …