[HTML][HTML] Lysosomal disorders: from storage to cellular damage

A Ballabio, V Gieselmann - Biochimica et Biophysica Acta (BBA)-Molecular …, 2009 - Elsevier
Lysosomal storage diseases represent a group of about 50 genetic disorders caused by
deficiencies of lysosomal and non-lysosomal proteins. Patients accumulate compounds …

The life, death, and replacement of oligodendrocytes in the adult CNS

DM McTigue, RB Tripathi - Journal of neurochemistry, 2008 - Wiley Online Library
Oligodendrocytes (OLs) are mature glial cells that myelinate axons in the brain and spinal
cord. As such, they are integral to functional and efficient neuronal signaling. The embryonic …

Ambroxol chaperone therapy for neuronopathic Gaucher disease: A pilot study

A Narita, K Shirai, S Itamura, A Matsuda… - Annals of clinical …, 2016 - Wiley Online Library
Objective Gaucher disease (GD) is a lysosomal storage disease characterized by a
deficiency of glucocerebrosidase. Although enzyme‐replacement and substrate‐reduction …

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 …

Modulation of inflammation in brain: a matter of fat

AA Farooqui, LA Horrocks… - Journal of …, 2007 - Wiley Online Library
Neuroinflammation is a host defense mechanism associated with neutralization of an insult
and restoration of normal structure and function of brain. Neuroinflammation is a hallmark of …

Metformin attenuated the autoimmune disease of the central nervous system in animal models of multiple sclerosis

N Nath, M Khan, MK Paintlia, MN Hoda… - The Journal of …, 2009 - journals.aai.org
Experimental autoimmune encephalomyelitis (EAE) is a T cell-mediated autoimmune
disease of the CNS. Metformin is the most widely used drug for diabetes and mediates its …

Nanoceria: a rare-earth nanoparticle as a novel anti-angiogenic therapeutic agent in ovarian cancer

S Giri, A Karakoti, RP Graham, JL Maguire, CM Reilly… - PloS one, 2013 - journals.plos.org
Ovarian cancer (OvCa) is the fifth most common cause of death from all cancers among
women in United Sates and the leading cause of death from gynecological malignancies …

[HTML][HTML] Value of glucosylsphingosine (Lyso-Gb1) as a biomarker in Gaucher disease: a systematic literature review

S Revel-Vilk, M Fuller, A Zimran - International journal of molecular …, 2020 - mdpi.com
The challenges in the diagnosis, prognosis, and monitoring of Gaucher disease (GD), an
autosomal recessive inborn error of glycosphingolipid metabolism, can negatively impact …

The cellular pathology of lysosomal diseases

TM Cox, MB Cachón‐González - The Journal of pathology, 2012 - Wiley Online Library
With a constitutive recycling function and the capacity to digest exogenous material as well
as endogenous organelles in the process of autophagy, lysosomes are at the heart of the …

An early glycolysis burst in microglia regulates mitochondrial dysfunction in oligodendrocytes under neuroinflammation

H Suhail, M Nematullah, F Rashid, M Sajad, M Fatma… - Iscience, 2023 - cell.com
Metabolism and energy processes governing oligodendrocyte function during
neuroinflammatory disease are of great interest. However, how varied cellular environments …