(Q36302113)

English

Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3Δex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease).

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scholarly article

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Europe PubMed Central

PMC publication ID

13 August 2017

Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3Δex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease). (English)

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

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juvenile neuronal ceroid lipofuscinosis

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photoreceptor protein

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Ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease)

1 reference

GOA release 2020-03-11

Daniel F Cutler

object named as

Daniel F Cutler

5

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Clare E Futter

6

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Miguel C. Seabra

object named as

Miguel C Seabra

4

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author name string

Silène T Wavre-Shapton

1

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Europe PubMed Central

PMC publication ID

13 August 2017

Alessandra A Calvi

2

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Europe PubMed Central

PMC publication ID

13 August 2017

Mark Turmaine

3

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Europe PubMed Central

PMC publication ID

13 August 2017

Hannah M Mitchison

7

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Europe PubMed Central

PMC publication ID

13 August 2017

language of work or name

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publication date

8 October 2015

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Europe PubMed Central

PMC publication ID

13 August 2017

Human Molecular Genetics

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Europe PubMed Central

PMC publication ID

13 August 2017

24

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Europe PubMed Central

PMC publication ID

13 August 2017

24

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

7060-7074

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

describes a project that uses

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Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4654058/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

Autophagosome dynamics in neurodegeneration at a glance

1 reference

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1 reference

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30 September 2017

Phagosome maturation during endosome interaction revealed by partial rhodopsin processing in retinal pigment epithelium

1 reference

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FRET-assisted determination of CLN3 membrane topology

1 reference

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Non-invasive assessment of retinal alterations in mouse models of infantile and juvenile neuronal ceroid lipofuscinosis by spectral domain optical coherence tomography

1 reference

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Novel CLN3 mutation causing autophagic vacuolar myopathy

1 reference

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1 reference

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CLN3 deficient cells display defects in the ARF1-Cdc42 pathway and actin-dependent events

1 reference

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CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells

1 reference

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Noncanonical autophagy promotes the visual cycle

1 reference

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Neuronal ceroid lipofuscinosis: impact of recent genetic advances and expansion of the clinicopathologic spectrum

1 reference

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30 September 2017

Signals from the lysosome: a control centre for cellular clearance and energy metabolism

1 reference

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30 September 2017

Alterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts

1 reference

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30 September 2017

Cell biology and function of neuronal ceroid lipofuscinosis-related proteins

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Mouse models of neuronal ceroid lipofuscinoses: useful pre-clinical tools to delineate disease pathophysiology and validate therapeutics

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartments

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Morphologic and functional correlates of synaptic pathology in the cathepsin D knockout mouse model of congenital neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

The yeast Batten disease orthologue Btn1 controls endosome-Golgi retrograde transport via SNARE assembly

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3(Δex7/8)-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Autophagy gone awry in neurodegenerative diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Retinal pigment epithelium defects accelerate photoreceptor degeneration in cell type-specific knockout mouse models of choroideremia

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Juvenile neuronal ceroid lipofuscinosis (JNCL) and the eye.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Pathogenic cascades in lysosomal disease-Why so complex?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Transcript and in silico analysis of CLN3 in juvenile neuronal ceroid lipofuscinosis and associated mouse models

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Autophagy in the Pathogenesis of Disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

The neuronal ceroid-lipofuscinoses: from past to present

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Batten disease: features to facilitate early diagnosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

The molecular regulation of organelle transport in mammalian retinal pigment epithelial cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Saccharomyces cerevisiae lacking Btn1p modulate vacuolar ATPase activity to regulate pH imbalance in the vacuole

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

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Mouse models of age-related macular degeneration

1 reference

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30 September 2017

Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

The retinal pigment epithelium in visual function

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

AP-1 and AP-3 facilitate lysosomal targeting of Batten disease protein CLN3 via its dileucine motif.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Interconnections of CLN3, Hook1 and Rab proteins link Batten disease to defects in the endocytic pathway

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Selectivity and types of cell death in the neuronal ceroid lipofuscinoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

A role in vacuolar arginine transport for yeast Btn1p and for human CLN3, the protein defective in Batten disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Abnormal phagocytosis by retinal pigmented epithelium that lacks myosin VIIa, the Usher syndrome 1B protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Progressive age-related changes similar to age-related macular degeneration in a transgenic mouse model

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

CLN3 protein is targeted to neuronal synapses but excluded from synaptic vesicles: new clues to Batten disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Rab5 regulates the kiss and run fusion between phagosomes and endosomes and the acquisition of phagosome leishmanicidal properties in RAW 264.7 macrophages

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Vision loss as the presenting sign in juvenile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

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Mutation of the receptor tyrosine kinase gene Mertk in the retinal dystrophic RCS rat

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected]

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Myosin VIIa participates in opsin transport through the photoreceptor cilium

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Identification of two distinct mechanisms of phagocytosis controlled by different Rho GTPases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

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Batten disease and mitochondrial pathways of proteolysis

1 reference

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30 September 2017

Multivesicular endosomes containing internalized EGF-EGF receptor complexes mature and then fuse directly with lysosomes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Juvenile Batten's disease: an ophthalmological assessment of 26 patients

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

Participation of the retinal pigment epithelium in the rod outer segment renewal process

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

30 September 2017

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1 reference

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30 September 2017

Novel interactions of CLN3 protein link Batten disease to dysregulation of fodrin-Na+, K+ ATPase complex.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

29 June 2018

LAMP proteins are required for fusion of lysosomes with phagosomes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

29 June 2018

Biogenesis of phagolysosomes proceeds through a sequential series of interactions with the endocytic apparatus

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

29 June 2018

Phagosome degradation in the tapetal retinal pigment epithelium of the opossum

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

29 June 2018

Phagosome movement and the diurnal pattern of phagocytosis in the tapetal retinal pigment epithelium of the opossum

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

29 June 2018

The epidemiology of progressive intellectual and neurological deterioration in childhood.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

S. pombe btn1, the orthologue of the Batten disease gene CLN3, is required for vacuole protein sorting of Cpy1p and Golgi exit of Vps10p

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

Loss of the Batten disease gene CLN3 prevents exit from the TGN of the mannose 6-phosphate receptor.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

C-terminal prenylation of the CLN3 membrane glycoprotein is required for efficient endosomal sorting to lysosomes.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease).

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

Mouse gene knockout models for the CLN2 and CLN3 forms of ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

Phagocytosis of outer segments by retinal pigment epithelium: phagosome-lysosome interaction

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4654058

22 September 2018

Guidelines for the use and interpretation of assays for monitoring autophagy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV406

21 January 2018

A knock-in reporter model of Batten disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/26450516

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lysosomal storage diseases as disorders of autophagy

1 reference

https://pubmed.ncbi.nlm.nih.gov/26450516

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cln3(Deltaex7/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth

1 reference

https://pubmed.ncbi.nlm.nih.gov/26450516

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The distribution of actin in cultured normal and dystrophic rat pigment epithelial cells during the phagocytosis of rod outer segments

1 reference

https://pubmed.ncbi.nlm.nih.gov/26450516

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Histopathologic and immunocytochemical analysis of the retina and ocular tissues in Batten disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/26450516

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1093/HMG/DDV406

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

ResearchGate publication ID

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