Presentation is loading. Please wait.

Presentation is loading. Please wait.

Huntington’s Disease: Nuclear Gatekeepers Under Attack

Published byDewi Sudjarwadi Modified over 5 years ago

Similar presentations

Presentation on theme: "Huntington’s Disease: Nuclear Gatekeepers Under Attack"— Presentation transcript:

1 Huntington’s Disease: Nuclear Gatekeepers Under Attack
Matthew B. Veldman, X. William Yang  Neuron  Volume 94, Issue 1, Pages 1-4 (April 2017) DOI: /j.neuron Copyright © Terms and Conditions

2 Figure 1 The Balance of Passive and Active Nuclear Transport Is Disrupted in Aging and Huntington’s Disease Youthful neurons (left) exhibit a passive permeability for proteins of <40 kDa, helping to maintain a segregation of cytoplasmic, MAP2, and nuclear Ran-GTP proteins. mRNA is efficiently transported out of the nucleus. In aging neurons (center), the nuclear envelope and lamina begin to fold, pore permeability is increased, mRNA accumulates in the nucleus, and RanGAP1 aggregates on the perinuclear surface. During Huntington’s disease (right), mutant Huntingtin (mHTT) aggregates in the nucleus, partially sequestering nuclear pore components NUP62, Gle1, and RanGAP1. Very large RanGAP1 perinuclear aggregates are observed, and the nuclear envelope and lamina is severely folded. Passive permeability is compromised, allowing cytoplasmic proteins such as MAP2 to leak in, and active transport is decreased, such that Ran-GTP and Ran-GDP accumulate in the cytoplasm and mRNA remains in the nucleus. Neuron  , 1-4DOI: ( /j.neuron ) Copyright © Terms and Conditions

Download ppt "Huntington’s Disease: Nuclear Gatekeepers Under Attack"

Similar presentations

The Neurobiology of Decision: Consensus and Controversy Joseph W. Kable, Paul W. Glimcher Neuron Volume 63, Issue 6, Pages 733-745 (September 2009) DOI:

The Neurobiology of Decision: Consensus and Controversy Joseph W. Kable, Paul W. Glimcher Neuron Volume 63, Issue 6, Pages (September 2009) DOI:
Irreplaceability of Neuronal Ensembles after Memory Allocation Naoki Matsuo Cell Reports Volume 11, Issue 3, Pages 351-357 (April 2015) DOI: 10.1016/j.celrep.2015.03.042.

Irreplaceability of Neuronal Ensembles after Memory Allocation Naoki Matsuo Cell Reports Volume 11, Issue 3, Pages (April 2015) DOI: /j.celrep
Treating the Developing versus Developed Brain: Translating Preclinical Mouse and Human Studies B.J. Casey, Charles E. Glatt, Francis S. Lee Neuron Volume.

Treating the Developing versus Developed Brain: Translating Preclinical Mouse and Human Studies B.J. Casey, Charles E. Glatt, Francis S. Lee Neuron Volume.
Rapid Encoding of New Memories by Individual Neurons in the Human Brain Matias J. Ison, Rodrigo Quian Quiroga, Itzhak Fried Neuron Volume 87, Issue 1,

Rapid Encoding of New Memories by Individual Neurons in the Human Brain Matias J. Ison, Rodrigo Quian Quiroga, Itzhak Fried Neuron Volume 87, Issue 1,
Preference Distributions of Primary Motor Cortex Neurons Reflect Control Solutions Optimized for Limb Biomechanics Timothy P. Lillicrap, Stephen H. Scott.

Preference Distributions of Primary Motor Cortex Neurons Reflect Control Solutions Optimized for Limb Biomechanics Timothy P. Lillicrap, Stephen H. Scott.
The Pathobiology of Vascular Dementia Costantino Iadecola Neuron Volume 80, Issue 4, Pages 844-866 (November 2013) DOI: 10.1016/j.neuron.2013.10.008 Copyright.

The Pathobiology of Vascular Dementia Costantino Iadecola Neuron Volume 80, Issue 4, Pages (November 2013) DOI: /j.neuron Copyright.
Intensity-Dependent Adaptation of Cortical and Thalamic Neurons Is Controlled by Brainstem Circuits of the Sensory Pathway Elad Ganmor, Yonatan Katz, Ilan.

Intensity-Dependent Adaptation of Cortical and Thalamic Neurons Is Controlled by Brainstem Circuits of the Sensory Pathway Elad Ganmor, Yonatan Katz, Ilan.
Decision Making as a Window on Cognition Michael N. Shadlen, Roozbeh Kiani Neuron Volume 80, Issue 3, Pages 791-806 (October 2013) DOI: 10.1016/j.neuron.2013.10.047.

Decision Making as a Window on Cognition Michael N. Shadlen, Roozbeh Kiani Neuron Volume 80, Issue 3, Pages (October 2013) DOI: /j.neuron
Contour Saliency in Primary Visual Cortex Wu Li, Valentin Piëch, Charles D. Gilbert Neuron Volume 50, Issue 6, Pages 951-962 (June 2006) DOI: 10.1016/j.neuron.2006.04.035.

Contour Saliency in Primary Visual Cortex Wu Li, Valentin Piëch, Charles D. Gilbert Neuron Volume 50, Issue 6, Pages (June 2006) DOI: /j.neuron
Functional Analysis of the Neurofibromatosis Type 2 Protein by Means of Disease- Causing Point Mutations Renee P. Stokowski, David R. Cox The American.

Functional Analysis of the Neurofibromatosis Type 2 Protein by Means of Disease- Causing Point Mutations Renee P. Stokowski, David R. Cox The American.
Neuronal Cell Types and Connectivity: Lessons from the Retina H. Sebastian Seung, Uygar Sümbül Neuron Volume 83, Issue 6, Pages 1262-1272 (September 2014)

Neuronal Cell Types and Connectivity: Lessons from the Retina H. Sebastian Seung, Uygar Sümbül Neuron Volume 83, Issue 6, Pages (September 2014)
Molecular Motors in Neurons: Transport Mechanisms and Roles in Brain Function, Development, and Disease Nobutaka Hirokawa, Shinsuke Niwa, Yosuke Tanaka.

Molecular Motors in Neurons: Transport Mechanisms and Roles in Brain Function, Development, and Disease Nobutaka Hirokawa, Shinsuke Niwa, Yosuke Tanaka.
Reinstating Aberrant mTORC1 Activity in Huntington’s Disease Mice Improves Disease Phenotypes John H. Lee, Luis Tecedor, Yong Hong Chen, Alex Mas Monteys,

Reinstating Aberrant mTORC1 Activity in Huntington’s Disease Mice Improves Disease Phenotypes John H. Lee, Luis Tecedor, Yong Hong Chen, Alex Mas Monteys,
Whole-Brain Mapping of Direct Inputs to Midbrain Dopamine Neurons Mitsuko Watabe-Uchida, Lisa Zhu, Sachie K. Ogawa, Archana Vamanrao, Naoshige Uchida Neuron.

Whole-Brain Mapping of Direct Inputs to Midbrain Dopamine Neurons Mitsuko Watabe-Uchida, Lisa Zhu, Sachie K. Ogawa, Archana Vamanrao, Naoshige Uchida Neuron.
Pyramidal Neurons Grow Up and Change Their Mind Gord Fishell, Carina Hanashima Neuron Volume 57, Issue 3, Pages 333-338 (February 2008) DOI: 10.1016/j.neuron.2008.01.018.

Pyramidal Neurons Grow Up and Change Their Mind Gord Fishell, Carina Hanashima Neuron Volume 57, Issue 3, Pages (February 2008) DOI: /j.neuron
Hippocampal Activity Patterns Carry Information about Objects in Temporal Context Liang-Tien Hsieh, Matthias J. Gruber, Lucas J. Jenkins, Charan Ranganath.

Hippocampal Activity Patterns Carry Information about Objects in Temporal Context Liang-Tien Hsieh, Matthias J. Gruber, Lucas J. Jenkins, Charan Ranganath.
Dissociable Medial Prefrontal Contributions to Judgments of Similar and Dissimilar Others Jason P. Mitchell, C. Neil Macrae, Mahzarin R. Banaji Neuron.

Dissociable Medial Prefrontal Contributions to Judgments of Similar and Dissimilar Others Jason P. Mitchell, C. Neil Macrae, Mahzarin R. Banaji Neuron.
2015 Alzheimer's disease facts and figures Alzheimer's & Dementia: The Journal of the Alzheimer's Association Volume 11, Issue 3, Pages 332-384 (March.

2015 Alzheimer's disease facts and figures Alzheimer's & Dementia: The Journal of the Alzheimer's Association Volume 11, Issue 3, Pages (March.
Mechanisms of Age-Related Macular Degeneration Jayakrishna Ambati, Benjamin J. Fowler Neuron Volume 75, Issue 1, Pages 26-39 (July 2012) DOI: 10.1016/j.neuron.2012.06.018.

Mechanisms of Age-Related Macular Degeneration Jayakrishna Ambati, Benjamin J. Fowler Neuron Volume 75, Issue 1, Pages (July 2012) DOI: /j.neuron
Adaptation to Natural Binocular Disparities in Primate V1 Explained by a Generalized Energy Model Ralf M. Haefner, Bruce G. Cumming Neuron Volume 57, Issue.

Adaptation to Natural Binocular Disparities in Primate V1 Explained by a Generalized Energy Model Ralf M. Haefner, Bruce G. Cumming Neuron Volume 57, Issue.

Similar presentations

Ads by Google