Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

The FTLD Risk Factor TMEM106B Regulates the Transport of Lysosomes at the Axon Initial Segment of Motoneurons

(2020) Patrick Lüningschrör et al.   Cell Reports

Loss of TMEM106B leads to myelination deficits: implications for frontotemporal dementia treatment strategies

(2020) Xiaolai Zhou et al.   BRAIN

A role of the frontotemporal lobar degeneration risk factor TMEM106B in myelination

(2020) Tuancheng Feng et al.   BRAIN

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

A recurrent TMEM106B mutation in hypomyelinating leukodystrophy: A rapid diagnostic assay

(2020) Satoru Ikemoto et al.   BRAIN & DEVELOPMENT

Opposite microglial activation stages upon loss of PGRN or TREM2 result in reduced cerebral glucose metabolism

(2019) Julia K Götzl et al.   EMBO Molecular Medicine

The TMEM106B FTLD-protective variant, rs1990621, is also associated with increased neuronal proportion

(2019) Zeran Li et al.   ACTA NEUROPATHOLOGICA

Partial Tmem106b reduction does not correct abnormalities due to progranulin haploinsufficiency

(2018) Andrew E. Arrant et al.   Molecular Neurodegeneration

TMEM106B haplotypes have distinct gene expression patterns in aged brain

(2018) Yingxue Ren et al.   Molecular Neurodegeneration

Coupling between physiological TSPO expression in brain and myocardium allows stabilization of late-phase cerebral [ 18 F]GE180 PET quantification

(2018) Maximilian Deussing et al.   NEUROIMAGE

Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity

(2018) Alexandra M. Nicholson et al.   Acta Neuropathologica Communications

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

(2018) Julia K. Götzl et al.   Molecular Neurodegeneration

Frontotemporal dementia causative CHMP2B impairs neuronal endolysosomal traffic-rescue by TMEM106B knockdown

(2018) Emma L Clayton et al.   BRAIN

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

(2017) Sander Beel et al.   HUMAN MOLECULAR GENETICS

Progranulin deficiency causes impairment of autophagy and TDP-43 accumulation

(2017) Michael C. Chang et al.   JOURNAL OF EXPERIMENTAL MEDICINE

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

Loss of TMEM106B Ameliorates Lysosomal and Frontotemporal Dementia-Related Phenotypes in Progranulin-Deficient Mice

(2017) Zoe A. Klein et al.   NEURON

Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis

(2017) Michael E. Ward et al.   Science Translational Medicine

Differential Aging Analysis in Human Cerebral Cortex Identifies Variants in TMEM106B and GRN that Regulate Aging Phenotypes

(2017) Herve Rhinn et al.   Cell Systems

Impaired protein degradation in FTLD and related disorders

(2016) Julia K. Götzl et al.   AGEING RESEARCH REVIEWS

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

(2016) Hansen Lui et al.   CELL

TREM2 deficiency reduces the efficacy of immunotherapeutic amyloid clearance

(2016) Xianyuan Xiang et al.   EMBO Molecular Medicine

Glial Activation and Glucose Metabolism in a Transgenic Amyloid Mouse Model: A Triple-Tracer PET Study

(2016) M. Brendel et al.   JOURNAL OF NUCLEAR MEDICINE

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

C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD

(2016) Yuanjing Liu et al.   NEURON

Core neuropathological abnormalities in progranulin-deficient mice are penetrant on multiple genetic backgrounds

(2016) T.L. Petkau et al.   NEUROSCIENCE

Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS–FTD-linkedUBQLN2mutations

(2016) Nhat T. T. Le et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prosaposin facilitates sortilin-independent lysosomal trafficking of progranulin

(2015) Xiaolai Zhou et al.   JOURNAL OF CELL BIOLOGY

Reassessment of Risk Genotypes (GRN,TMEM106B, andABCC9Variants) Associated With Hippocampal Sclerosis of Aging Pathology

(2015) Peter T. Nelson et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

The TMEM106B locus and TDP-43 pathology in older persons without FTLD

(2015) L. Yu et al.   NEUROLOGY

Differential clinicopathologic and genetic features of late-onset amnestic dementias

(2014) Melissa E. Murray et al.   ACTA NEUROPATHOLOGICA

TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions

(2014) Michael D. Gallagher et al.   ACTA NEUROPATHOLOGICA

TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia

(2014) Marka van Blitterswijk et al.   ACTA NEUROPATHOLOGICA

Lysosome size, motility and stress response regulated by fronto-temporal dementia modifier TMEM106B

(2014) Massimiliano Stagi et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

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

(2014) Yoshinori Tanaka et al.   Acta Neuropathologica Communications

NCL disease mechanisms

(2013) David N. Palmer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The FTLD risk factor TMEM106B and MAP6 control dendritic trafficking of lysosomes

(2013) Benjamin M. Schwenk et al.   EMBO JOURNAL

Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not

(2013) John Hardy et al.   EXPERIMENTAL NEUROLOGY

TMEM106B p.T185S regulates TMEM106B protein levels: implications for frontotemporal dementia

(2013) Alexandra M. Nicholson et al.   JOURNAL OF NEUROCHEMISTRY

The role of autophagy in neurodegenerative disease

(2013) Ralph A Nixon   NATURE MEDICINE

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

The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function

(2012) Owen A. Brady et al.   HUMAN MOLECULAR GENETICS

Membrane Orientation and Subcellular Localization of Transmembrane Protein 106B (TMEM106B), a Major Risk Factor for Frontotemporal Lobar Degeneration

(2012) Christina M. Lang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TMEM106B, the Risk Gene for Frontotemporal Dementia, Is Regulated by the microRNA-132/212 Cluster and Affects Progranulin Pathways

(2012) A. S. Chen-Plotkin et al.   JOURNAL OF NEUROSCIENCE

Cellular ageing, increased mortality and FTLD-TDP-associated neuropathology in progranulin knockout mice

(2012) Hans Wils et al.   JOURNAL OF PATHOLOGY

Mechanisms of Granulin Deficiency: Lessons from Cellular and Animal Models

(2012) Gernot Kleinberger et al.   MOLECULAR NEUROBIOLOGY

TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease

(2012) N. J. Rutherford et al.   NEUROLOGY

Association of TMEM106B Gene Polymorphism With Age at Onset in Granulin Mutation Carriers and Plasma Granulin Protein Levels

(2011) Carlos Cruchaga et al.   ARCHIVES OF NEUROLOGY

TFEB regulates autophagy: An integrated coordination of cellular degradation and recycling processes

(2011) Carmine Settembre et al.   Autophagy

TMEM106B is associated with frontotemporal lobar degeneration in a clinically diagnosed patient cohort

(2011) Julie van der Zee et al.   BRAIN

Rescue of Progranulin Deficiency Associated with Frontotemporal Lobar Degeneration by Alkalizing Reagents and Inhibition of Vacuolar ATPase

(2011) A. Capell et al.   JOURNAL OF NEUROSCIENCE

Synaptic dysfunction in progranulin-deficient mice

(2011) Terri L. Petkau et al.   NEUROBIOLOGY OF DISEASE

Core features of frontotemporal dementia recapitulated in progranulin knockout mice

(2011) N. Ghoshal et al.   NEUROBIOLOGY OF DISEASE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Transcriptional gene network inference from a massive dataset elucidates transcriptome organization and gene function

(2011) Vincenzo Belcastro et al.   NUCLEIC ACIDS RESEARCH

TFEB Links Autophagy to Lysosomal Biogenesis

(2011) C. Settembre et al.   SCIENCE

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

Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures

(2010) Aobo Guo et al.   BRAIN RESEARCH

Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

(2010) Fangfang Yin et al.   FASEB JOURNAL

Increased caspase activation and decreased TDP-43 solubility in progranulin knockout cortical cultures

(2010) Gernot Kleinberger et al.   JOURNAL OF NEUROCHEMISTRY

Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions

(2010) Vivianna M Van Deerlin et al.   NATURE GENETICS

TMEM106B regulates progranulin levels and the penetrance of FTLD in GRN mutation carriers

(2010) N. Finch et al.   NEUROLOGY

Sortilin-Mediated Endocytosis Determines Levels of the Frontotemporal Dementia Protein, Progranulin

(2010) Fenghua Hu et al.   NEURON

TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration

(2010) Hans Wils et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

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

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

A Gene Network Regulating Lysosomal Biogenesis and Function

(2009) Marco Sardiello et al.   SCIENCE

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2008) Masato Hasegawa et al.   ANNALS OF NEUROLOGY