Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD

White matter aging drives microglial diversity

(2021) Shima Safaiyan et al.   NEURON

TREM2 Regulates Microglial Cholesterol Metabolism upon Chronic Phagocytic Challenge

(2020) Alicia A. Nugent et al.   NEURON

Loss of TMEM 106B and PGRN leads to severe lysosomal abnormalities and neurodegeneration in mice

(2020) Tuancheng Feng et al.   EMBO REPORTS

Loss of Tmem106b exacerbates FTLD pathologies and causes motor deficits in progranulin‐deficient mice

(2020) Xiaolai Zhou et al.   EMBO REPORTS

Discovery of small molecules that normalize the transcriptome and enhance cysteine cathepsin activity in progranulin-deficient microglia

(2020) Maria A. Telpoukhovskaia et al.   Scientific Reports

Neurotoxic microglia promote TDP-43 proteinopathy in progranulin deficiency

(2020) Jiasheng Zhang et al.   NATURE

Iba-1-/CD68+ microglia are a prominent feature of age-associated deep subcortical white matter lesions

(2019) Rachel Waller et al.   PLoS One

Progranulin deficiency leads to reduced glucocerebrosidase activity

(2019) Xiaolai Zhou et al.   PLoS One

The lysosomal function of progranulin, a guardian against neurodegeneration

(2018) Daniel H. Paushter et al.   ACTA NEUROPATHOLOGICA

Global and Widespread Local White Matter Abnormalities in Juvenile Neuronal Ceroid Lipofuscinosis

(2018) U. Roine et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Lifelong cortical myelin plasticity and age-related degeneration in the live mammalian brain

(2018) Robert A. Hill et al.   NATURE NEUROSCIENCE

Defective cholesterol clearance limits remyelination in the aged central nervous system

(2018) Ludovico Cantuti-Castelvetri et al.   SCIENCE

Pathological correlates of white matter hyperintensities in a case of progranulin mutation associated frontotemporal dementia

(2018) Ione O.C. Woollacott et al.   NEUROCASE

Review: Neuropathology of non-tau frontotemporal lobar degeneration

(2018) M. Neumann et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Progranulin-mediated deficiency of cathepsin D results in FTD and NCL-like phenotypes in neurons derived from FTD patients

(2017) Clarissa Valdez et al.   HUMAN MOLECULAR GENETICS

Progranulin functions as a cathepsin D chaperone to stimulate axonal outgrowth in vivo

(2017) Sander Beel et al.   HUMAN MOLECULAR GENETICS

Myelin as an inflammatory mediator: Myelin interactions with complement, macrophages, and microglia in spinal cord injury

(2017) Timothy J. Kopper et al.   JOURNAL OF NEUROSCIENCE RESEARCH

The lysosomal protein cathepsin L is a progranulin protease

(2017) Chris W. Lee et al.   Molecular Neurodegeneration

Lysosomal processing of progranulin

(2017) Xiaolai Zhou et al.   Molecular Neurodegeneration

Progranulin, lysosomal regulation and neurodegenerative disease

(2017) Aimee W. Kao et al.   NATURE REVIEWS NEUROSCIENCE

White matter hyperintensities are seen only in GRN mutation carriers in the GENFI cohort

(2017) Carole H. Sudre et al.   NeuroImage-Clinical

Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation

(2016) Hansen Lui et al.   CELL

Age-related myelin degradation burdens the clearance function of microglia during aging

(2016) Shima Safaiyan et al.   NATURE NEUROSCIENCE

Portuguese family with the co-occurrence of frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis phenotypes due to progranulin gene mutation

(2016) Maria R. Almeida et al.   NEUROBIOLOGY OF AGING

Complement Protein C1q Modulates Neurite Outgrowth In Vitro and Spinal Cord Axon Regeneration In Vivo

(2015) S. L. Peterson et al.   JOURNAL OF NEUROSCIENCE

Myelination of the Nervous System: Mechanisms and Functions

(2014) Klaus-Armin Nave et al.   Annual Review of Cell and Developmental Biology

Extensive White Matter Involvement in Patients With Frontotemporal Lobar Degeneration

(2014) Paola Caroppo et al.   JAMA Neurology

Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice

(2014) Yoshinori Tanaka et al.   Acta Neuropathologica Communications

Inflammatory components in human Alzheimer’s disease and after active amyloid-β42 immunization

(2013) Elina Zotova et al.   BRAIN

Identification of Long-Lived Proteins Reveals Exceptional Stability of Essential Cellular Structures

(2013) Brandon H. Toyama et al.   CELL

Increased lysosomal biogenesis in activated microglia and exacerbated neuronal damage after traumatic brain injury in progranulin-deficient mice

(2013) Y. Tanaka et al.   NEUROSCIENCE

Strikingly Different Clinicopathological Phenotypes Determined by Progranulin-Mutation Dosage

(2012) Katherine R. Smith et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Assessment of Disease Severity in Late Infantile Neuronal Ceroid Lipofuscinosis Using Multiparametric MR Imaging

(2012) J.P. Dyke et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury

(2012) Lauren Herl Martens et al.   JOURNAL OF CLINICAL INVESTIGATION

A harmonized classification system for FTLD-TDP pathology

(2011) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Accelerated Lipofuscinosis and Ubiquitination in Granulin Knockout Mice Suggest a Role for Progranulin in Successful Aging

(2010) Zeshan Ahmed et al.   AMERICAN JOURNAL OF PATHOLOGY

Distinct profiles of brain atrophy in frontotemporal lobar degeneration caused by progranulin and tau mutations

(2010) Jonathan D. Rohrer et al.   NEUROIMAGE

Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice

(2009) Fangfang Yin et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Recent insights into the molecular genetics of dementia

(2009) Rosa Rademakers et al.   TRENDS IN NEUROSCIENCES

Mutations in progranulin (GRN) within the spectrum of clinical and pathological phenotypes of frontotemporal dementia

(2008) John C van Swieten et al.   LANCET NEUROLOGY

Prominent phenotypic variability associated with mutations in Progranulin

(2007) Brendan J. Kelley et al.   NEUROBIOLOGY OF AGING