[HTML][HTML] Cell biology of the NCL proteins: what they do and don't do

J Cárcel-Trullols, AD Kovács, DA Pearce - Biochimica et Biophysica Acta …, 2015 - Elsevier
The fatal, primarily childhood neurodegenerative disorders, neuronal ceroid lipofuscinoses
(NCLs), are currently associated with mutations in 13 genes. The protein products of these …

Altered protein secretion in Batten disease

RJ Huber - Disease Models & Mechanisms, 2021 - journals.biologists.com
The neuronal ceroid lipofuscinoses (NCLs), collectively known as Batten disease, are a
group of neurological diseases that affect all ages and ethnicities worldwide. There are 13 …

Dictyostelium discoideum as a non‐mammalian biomedical model

J Martín‐González, JF Montero‐Bullón… - Microbial …, 2021 - Wiley Online Library
Dictyostelium discoideum is one of eight non‐mammalian model organisms recognized by
the National Institute of Health for the study of human pathology. The use of this slime mould …

CLN3, at the crossroads of endocytic trafficking

SL Cotman, S Lefrancois - Neuroscience letters, 2021 - Elsevier
The CLN3 gene was identified over two decades ago, but the primary function of the CLN3
protein remains unknown. Recessive inheritance of loss of function mutations in CLN3 are …

Cln5 is secreted and functions as a glycoside hydrolase in Dictyostelium

RJ Huber, S Mathavarajah - Cellular signalling, 2018 - Elsevier
Abstract Ceroid lipofuscinosis neuronal 5 (CLN5) is a member of a family of proteins that are
linked to neuronal ceroid lipofuscinosis (NCL). This devastating neurological disorder …

The past, present and future of Dictyostelium as a model system

S Bozzaro - The International Journal of Developmental Biology, 2019 - iris.unito.it
The social amoeba Dictyostelium discoideum has been a preferred model organism during
the last 50 years, particularly for the study of cell motility and chemotaxis, phagocytosis and …

[HTML][HTML] The contribution of multicellular model organisms to neuronal ceroid lipofuscinosis research

RJ Huber, SM Hughes, W Liu, A Morgan… - … et Biophysica Acta (BBA …, 2020 - Elsevier
The NCLs (neuronal ceroid lipofuscinosis) are forms of neurodegenerative disease that
affect people of all ages and ethnicities but are most prevalent in children. Commonly known …

[HTML][HTML] Autophagy in the neuronal ceroid lipofuscinoses (Batten disease)

WD Kim, MLDM Wilson-Smillie… - Frontiers in Cell and …, 2022 - frontiersin.org
The neuronal ceroid lipofuscinoses (NCLs), also referred to as Batten disease, are a family
of neurodegenerative diseases that affect all age groups and ethnicities around the globe. At …

[HTML][HTML] Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment …

Y Zhong, K Mohan, J Liu, A Al-Attar, P Lin… - … et Biophysica Acta (BBA …, 2020 - Elsevier
Juvenile neuronal ceroid lipofuscinosis (JNCL, aka. juvenile Batten disease or CLN3
disease) is a lysosomal storage disease characterized by progressive blindness, seizures …

[HTML][HTML] Aberrant Autophagy Impacts Growth and Multicellular Development in a Dictyostelium Knockout Model of CLN5 Disease

MD McLaren, S Mathavarajah, WD Kim… - Frontiers in Cell and …, 2021 - frontiersin.org
Mutations in CLN5 cause a subtype of neuronal ceroid lipofuscinosis (NCL) called CLN5
disease. While the precise role of CLN5 in NCL pathogenesis is not known, recent work …