Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartments (Q24302244)

vesicle transport along microtubule

GOA release 2020-03-11

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neuronal ceroid lipofuscinosis

GOA release 2020-03-11

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8

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author name string

Kristiina Uusi-Rauva

1

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Aija Kyttälä

2

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Rik van der Kant

3

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Jouni Vesa

4

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Kimmo Tanhuanpää

5

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Jacques Neefjes

6

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Vesa M Olkkonen

Cellular and Molecular Life Sciences

7

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language of work or name

English

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publication date

June 2012

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published in

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volume

69

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issue

12

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page(s)

2075-89

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exact match

https://scigraph.springernature.com/pub.10.1007/s00018-011-0913-1

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cites work

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Defective intracellular transport of CLN3 is the molecular basis of Batten disease (JNCL)

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

21 January 2018

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Two motifs target Batten disease protein CLN3 to lysosomes in transfected nonneuronal and neuronal cells

21 January 2018

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

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Isolation of a novel gene underlying batten disease, CLN3

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The neuronal ceroid-lipofuscinoses: from past to present

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

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A novel role of the Batten disease gene CLN3: association with BMP synthesis

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CLN3P, the Batten's disease protein, is a novel palmitoyl-protein Delta-9 desaturase.

21 January 2018

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btn1, the Schizosaccharomyces pombe homologue of the human Batten disease gene CLN3, regulates vacuole homeostasis.

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CLN3 protein regulates lysosomal pH and alters intracellular processing of Alzheimer's amyloid-beta protein precursor and cathepsin D in human cells

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

21 January 2018

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Action of BTN1, the yeast orthologue of the gene mutated in Batten disease

21 January 2018

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

21 January 2018

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Defective lysosomal arginine transport in juvenile Batten disease

21 January 2018

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A function retained by the common mutant CLN3 protein is responsible for the late onset of juvenile neuronal ceroid lipofuscinosis.

21 January 2018

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Interactions between the juvenile Batten disease gene, CLN3, and the Notch and JNK signalling pathways.

21 January 2018

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The Batten disease gene CLN3 is required for the response to oxidative stress.

21 January 2018

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Neuronal vulnerability of CLN3 deletion to calcium-induced cytotoxicity is mediated by calsenilin

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Interaction between Sdo1p and Btn1p in the Saccharomyces cerevisiae model for Batten disease

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A novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cells

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

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

21 January 2018

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

21 January 2018

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

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Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons

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

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Regulation of caveolin-1 membrane trafficking by the Na/K-ATPase.

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Assays for interaction between Rab7 and oxysterol binding protein related protein 1L (ORP1L).

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Dynein-mediated vesicle transport controls intracellular Salmonella replication

21 January 2018

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Activation of endosomal dynein motors by stepwise assembly of Rab7-RILP-p150Glued, ORP1L, and the receptor betalll spectrin

21 January 2018

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The Rab7 effector protein RILP controls lysosomal transport by inducing the recruitment of dynein-dynactin motors

21 January 2018

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Changes in lysosome shape and distribution correlated with changes in cytoplasmic pH

21 January 2018

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Kinesin-2 is a motor for late endosomes and lysosomes

21 January 2018

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A 30-year follow-up of a neuronal ceroid lipofuscinosis patient with mutations in CLN3 and protracted disease course.

21 January 2018

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Molecular characterization of the 50-kD subunit of dynactin reveals function for the complex in chromosome alignment and spindle organization during mitosis

21 January 2018

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Point mutation of adenosine triphosphate-binding motif generated rigor kinesin that selectively blocks anterograde lysosome membrane transport

21 January 2018

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Targeted disruption of mouse conventional kinesin heavy chain, kif5B, results in abnormal perinuclear clustering of mitochondria

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Structural basis for recruitment of RILP by small GTPase Rab7

21 January 2018

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Cholesterol sensor ORP1L contacts the ER protein VAP to control Rab7-RILP-p150 Glued and late endosome positioning

21 January 2018

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Identification of a transactivation motif in the CLN3 protein.

21 January 2018

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

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21 January 2018

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Rab7 regulates late endocytic trafficking downstream of multivesicular body biogenesis and cargo sequestration

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00018-011-0913-1

Role of the small GTPase Rab7 in the late endocytic pathway.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00018-011-0913-1

Rabring7, a novel Rab7 target protein with a RING finger motif

21 January 2018

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The oxysterol-binding protein homologue ORP1L interacts with Rab7 and alters functional properties of late endocytic compartments

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00018-011-0913-1

FYCO1 is a Rab7 effector that binds to LC3 and PI3P to mediate microtubule plus end-directed vesicle transport

21 January 2018

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Rubicon controls endosome maturation as a Rab7 effector

21 January 2018

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Rab proteins and endocytic trafficking: potential targets for therapeutic intervention

21 January 2018

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Rab5 and Rab7 control endocytic sorting along the axonal retrograde transport pathway

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Mutations in the small GTP-ase late endosomal protein RAB7 cause Charcot-Marie-Tooth type 2B neuropathy

21 January 2018

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Lysosomal activity associated with developmental axon pruning

21 January 2018

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Rab7 is required for the normal progression of the autophagic pathway in mammalian cells

21 January 2018

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Role for Rab7 in maturation of late autophagic vacuoles

21 January 2018

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Mutant Rab7 causes the accumulation of cathepsin D and cation-independent mannose 6-phosphate receptor in an early endocytic compartment

21 January 2018

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Isolation of a novel gene underlying Batten disease, CLN3. The International Batten Disease Consortium

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12 December 2020

Opposing motor activities of dynein and kinesin determine retention and transport of MHC class II-containing compartments

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Apoptosis as the mechanism of neurodegeneration in Batten's disease.

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The rab7 GTPase resides on a vesicular compartment connected to lysosomes

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12 December 2020