Identifying and verifying Huntington's disease subtypes: Clinical features, neuroimaging, and cytokine changes
ConclusionWe have identified three HD clusters via clinical manifestations with distinct biomarkers. Our data shed light on better understanding about the pathophysiology of HD. (Source: Brain and Behavior)
Source: Brain and Behavior - March 18, 2024 Category: Neurology Authors: Ling ‐Xiao Cao,
Jin‐Hui Yin,
Gang Du,
Qing Yang,
Yue Huang Tags: ORIGINAL ARTICLE Source Type: research
Revisiting the Mitochondrial Function and Communication in Neurodegenerative Diseases
Curr Pharm Des. 2024 Mar 12. doi: 10.2174/0113816128286655240304070740. Online ahead of print.ABSTRACTNeurodegenerative disorders are distinguished by the progressive loss of anatomically or physiologically relevant neural systems. Atypical mitochondrial morphology and metabolic malfunction are found in many neurodegenerative disorders. Alteration in mitochondrial function can occur as a result of aberrant mitochondrial DNA, altered nuclear enzymes that interact with mitochondria actively or passively, or due to unexplained reasons. Mitochondria are intimately linked to the Endoplasmic reticulum (ER), and ER-mitochondrial ...
Source: Current Pharmaceutical Design - March 14, 2024 Category: Drugs & Pharmacology Authors: Nitu L Wankhede Mayur B Kale Mohit D Umare Sanket Lokhande Aman B Upaganlawar Pranay Wal Brijesh G Taksande Milind J Umekar Prasanna Shama Khandige Bhupendra Singh Vandana Sadananda Seema Ramniwas Tapan Behl Source Type: research
Revisiting the Mitochondrial Function and Communication in Neurodegenerative Diseases
Curr Pharm Des. 2024 Mar 12. doi: 10.2174/0113816128286655240304070740. Online ahead of print.ABSTRACTNeurodegenerative disorders are distinguished by the progressive loss of anatomically or physiologically relevant neural systems. Atypical mitochondrial morphology and metabolic malfunction are found in many neurodegenerative disorders. Alteration in mitochondrial function can occur as a result of aberrant mitochondrial DNA, altered nuclear enzymes that interact with mitochondria actively or passively, or due to unexplained reasons. Mitochondria are intimately linked to the Endoplasmic reticulum (ER), and ER-mitochondrial ...
Source: Current Pharmaceutical Design - March 14, 2024 Category: Drugs & Pharmacology Authors: Nitu L Wankhede Mayur B Kale Mohit D Umare Sanket Lokhande Aman B Upaganlawar Pranay Wal Brijesh G Taksande Milind J Umekar Prasanna Shama Khandige Bhupendra Singh Vandana Sadananda Seema Ramniwas Tapan Behl Source Type: research
Huntingtin HTT1a is generated in a CAG repeat-length-dependent manner in human tissues
CONCLUSIONS: Our results show that HTT1a expression occurs throughout a wide range of tissues and likely with all CAG lengths. Our data from peripheral sample sources demonstrate that HTT1a is indeed generated throughout the body in a CAG repeat-length-dependent manner. Therefore, the levels of HTT1a might be a sensitive marker of disease state and/or progression and should be monitored over time, especially in clinical trials targeting HTT expression.PMID:38459427 | DOI:10.1186/s10020-024-00801-2 (Source: Molecular Medicine)
Source: Molecular Medicine - March 8, 2024 Category: Molecular Biology Authors: Franziska Hoschek Julia Natan Maximilian Wagner Kirupa Sathasivam Alshaimaa Abdelmoez Bj örn von Einem Gillian P Bates G Bernhard Landwehrmeyer Andreas Neueder Source Type: research
TYROBP/DAP12 knockout in Huntington ’s disease Q175 mice cell-autonomously decreases microglial expression of disease-associated genes and non-cell-autonomously mitigates astrogliosis and motor deterioration
Huntington ’s disease (HD) is a fatal neurodegenerative disorder caused by an expansion of the CAG trinucleotide repeat in the Huntingtin gene (HTT). Immune activation is abundant in the striatum of HD patients. D... (Source: Journal of Neuroinflammation)
Source: Journal of Neuroinflammation - March 8, 2024 Category: Neurology Authors: Jordi Creus-Muncunill, Jean Vianney Haure-Mirande, Daniele Mattei, Joanna Bons, Angie V. Ramirez, B. Wade Hamilton, Chuhyon Corwin, Sarah Chowdhury, Birgit Schilling, Lisa M. Ellerby and Michelle E. Ehrlich Tags: Research Source Type: research
Agmatine mitigates behavioral abnormalities and neurochemical dysregulation associated with 3-Nitropropionic acid-induced Huntington's disease in rats
Neurotoxicology. 2024 Mar 5:S0161-813X(24)00022-6. doi: 10.1016/j.neuro.2024.03.002. Online ahead of print.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative condition characterized by a severe motor incoordination, cognitive decline, and psychiatric complications. However, a definitive cure for this devastating disorder remains elusive. Agmatine, a biogenic amine, has gain attention for its reported neuromodulatory and neuroprotective properties. The present study was designed to examine the influence of agmatine on the behavioral, biochemical, and molecular aspects of HD in an animal model. A mitochondr...
Source: Neurotoxicology - March 7, 2024 Category: Neurology Authors: Raj Katariya Kartikey Mishra Shivkumar Sammeta Milind Umekar Nandkishor Kotagale Brijesh Taksande Source Type: research
Agmatine mitigates behavioral abnormalities and neurochemical dysregulation associated with 3-Nitropropionic acid-induced Huntington's disease in rats
Neurotoxicology. 2024 Mar 5:S0161-813X(24)00022-6. doi: 10.1016/j.neuro.2024.03.002. Online ahead of print.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative condition characterized by a severe motor incoordination, cognitive decline, and psychiatric complications. However, a definitive cure for this devastating disorder remains elusive. Agmatine, a biogenic amine, has gain attention for its reported neuromodulatory and neuroprotective properties. The present study was designed to examine the influence of agmatine on the behavioral, biochemical, and molecular aspects of HD in an animal model. A mitochondr...
Source: Neurotoxicology - March 7, 2024 Category: Neurology Authors: Raj Katariya Kartikey Mishra Shivkumar Sammeta Milind Umekar Nandkishor Kotagale Brijesh Taksande Source Type: research
Agmatine mitigates behavioral abnormalities and neurochemical dysregulation associated with 3-Nitropropionic acid-induced Huntington's disease in rats
Neurotoxicology. 2024 Mar 5:S0161-813X(24)00022-6. doi: 10.1016/j.neuro.2024.03.002. Online ahead of print.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative condition characterized by a severe motor incoordination, cognitive decline, and psychiatric complications. However, a definitive cure for this devastating disorder remains elusive. Agmatine, a biogenic amine, has gain attention for its reported neuromodulatory and neuroprotective properties. The present study was designed to examine the influence of agmatine on the behavioral, biochemical, and molecular aspects of HD in an animal model. A mitochondr...
Source: Neurotoxicology - March 7, 2024 Category: Neurology Authors: Raj Katariya Kartikey Mishra Shivkumar Sammeta Milind Umekar Nandkishor Kotagale Brijesh Taksande Source Type: research
Agmatine mitigates behavioral abnormalities and neurochemical dysregulation associated with 3-Nitropropionic acid-induced Huntington's disease in rats
Neurotoxicology. 2024 Mar 5:S0161-813X(24)00022-6. doi: 10.1016/j.neuro.2024.03.002. Online ahead of print.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative condition characterized by a severe motor incoordination, cognitive decline, and psychiatric complications. However, a definitive cure for this devastating disorder remains elusive. Agmatine, a biogenic amine, has gain attention for its reported neuromodulatory and neuroprotective properties. The present study was designed to examine the influence of agmatine on the behavioral, biochemical, and molecular aspects of HD in an animal model. A mitochondr...
Source: Neurotoxicology - March 7, 2024 Category: Neurology Authors: Raj Katariya Kartikey Mishra Shivkumar Sammeta Milind Umekar Nandkishor Kotagale Brijesh Taksande Source Type: research
Disentangling the neurobiological bases of temporal impulsivity in Huntington's disease
ConclusionsThe present findings highlight the importance of investigating the spectrum of temporal impulsivity in HD. As, while less prevalent than other psychiatric features, this symptom is still reported to significantly impact the quality of life of patients and caregivers. This study provides evidence that individual differences observed in temporal impulsivity may be explained by variability in limbic frontostriatal tracts, while shedding light on the role of sensitivity to reward in modulating impulsive behavior through the selection of immediate rewards. (Source: Brain and Behavior)
Source: Brain and Behavior - March 7, 2024 Category: Neurology Authors: Helena Pardina ‐Torner,
Audrey E. De Paepe,
Clara Garcia‐Gorro,
Nadia Rodriguez‐Dechicha,
Irene Vaquer,
Matilde Calopa,
Jesus Ruiz‐Idiago,
Celia Mareca,
Ruth de Diego‐Balaguer,
Estela Camara Tags: ORIGINAL ARTICLE Source Type: research
Assessment of Fitbit Charge 4 for sleep stage and heart rate monitoring against polysomnography and during home monitoring in Huntington ’s disease
Journal of Clinical Sleep Medicine, Ahead of Print. (Source: Journal of Clinical Sleep Medicine : JCSM)
Source: Journal of Clinical Sleep Medicine : JCSM - March 7, 2024 Category: Sleep Medicine Authors: Emer P. Doheny
Klavs Renerts
Andreas Braun
Esther Werth
Christian Baumann
Philipp Baumgartner
Philippa Morgan-Jones
Monica Busse
Madeleine M. Lowery
Hans H. Jung
1School of Electrical and Electronic Engineering, University College Dublin, Dublin, Ireland
Source Type: research
Ribosome Profiling and Mass Spectrometry Reveal Widespread Mitochondrial Translation Defects in a Striatal Cell Model of Huntington Disease
Mol Cell Proteomics. 2024 Mar 4:100746. doi: 10.1016/j.mcpro.2024.100746. Online ahead of print.ABSTRACTHuntington disease (HD) is caused by an expanded polyglutamine mutation in huntingtin (mHTT) that promotes prominent atrophy in the striatum and subsequent psychiatric, cognitive, and choreiform movements. Multiple lines of evidence point to an association between HD and aberrant striatal mitochondrial functions; however, the present knowledge about whether (or how) mitochondrial mRNA translation is differentially regulated in HD remains unclear. We found that protein synthesis is diminished in HD mitochondria compared t...
Source: Molecular and Cellular Proteomics : MCP - March 6, 2024 Category: Molecular Biology Authors: Sunayana Dagar Manish Sharma George Tsaprailis Catherina Scharager Tapia Gogce Crynen Preksha Sandipkumar Joshi Neelam Shahani Srinivasa Subramaniam Source Type: research
Ribosome Profiling and Mass Spectrometry Reveal Widespread Mitochondrial Translation Defects in a Striatal Cell Model of Huntington Disease
Mol Cell Proteomics. 2024 Mar 4:100746. doi: 10.1016/j.mcpro.2024.100746. Online ahead of print.ABSTRACTHuntington disease (HD) is caused by an expanded polyglutamine mutation in huntingtin (mHTT) that promotes prominent atrophy in the striatum and subsequent psychiatric, cognitive, and choreiform movements. Multiple lines of evidence point to an association between HD and aberrant striatal mitochondrial functions; however, the present knowledge about whether (or how) mitochondrial mRNA translation is differentially regulated in HD remains unclear. We found that protein synthesis is diminished in HD mitochondria compared t...
Source: Molecular and Cellular Proteomics : MCP - March 6, 2024 Category: Molecular Biology Authors: Sunayana Dagar Manish Sharma George Tsaprailis Catherina Scharager Tapia Gogce Crynen Preksha Sandipkumar Joshi Neelam Shahani Srinivasa Subramaniam Source Type: research
Ribosome Profiling and Mass Spectrometry Reveal Widespread Mitochondrial Translation Defects in a Striatal Cell Model of Huntington Disease
Mol Cell Proteomics. 2024 Mar 4:100746. doi: 10.1016/j.mcpro.2024.100746. Online ahead of print.ABSTRACTHuntington disease (HD) is caused by an expanded polyglutamine mutation in huntingtin (mHTT) that promotes prominent atrophy in the striatum and subsequent psychiatric, cognitive, and choreiform movements. Multiple lines of evidence point to an association between HD and aberrant striatal mitochondrial functions; however, the present knowledge about whether (or how) mitochondrial mRNA translation is differentially regulated in HD remains unclear. We found that protein synthesis is diminished in HD mitochondria compared t...
Source: Molecular and Cellular Proteomics : MCP - March 6, 2024 Category: Molecular Biology Authors: Sunayana Dagar Manish Sharma George Tsaprailis Catherina Scharager Tapia Gogce Crynen Preksha Sandipkumar Joshi Neelam Shahani Srinivasa Subramaniam Source Type: research
