3.8 Review

SUMO-modifying Huntington's disease

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Article Biochemistry & Molecular Biology

Site-Specific Phosphorylation of Huntingtin Exon 1 Recombinant Proteins Enabled by the Discovery of Novel Kinases

Anass Chiki et al.

Summary: This study introduces a new and simple method for producing highly pure site-specifically phosphorylated Httex1 proteins. These advances are expected to expand the range of methods and experimental approaches for elucidating the mechanisms by which phosphorylation influences Httex1 or HTT structure, aggregation, interactome, and function(s) in health and disease.

CHEMBIOCHEM (2021)

Review Biochemistry & Molecular Biology

A Chain of Events: Regulating Target Proteins by SUMO Polymers

Nicolette S. Jansen et al.

Summary: SUMO polymers regulate various nuclear processes through dynamic signal transduction, including protein degradation. The fate of target proteins is influenced by the structure of SUMO polymers. Recent studies have demonstrated the diversity of SUMO polymer signal transduction for proteolytic and nonproteolytic purposes.

TRENDS IN BIOCHEMICAL SCIENCES (2021)

Article Multidisciplinary Sciences

PIAS1 modulates striatal transcription, DNA damage repair, and SUMOylation with relevance to Huntington's disease

Eva L. Morozko et al.

Summary: This study reveals the involvement of PIAS1 in DNA damage repair pathways and its connection with transcriptional modulation in neurodegenerative diseases. Knockdown of PIAS1 in HD mice normalizes transcriptional dysregulation and DNA damage repair mechanisms, impacting genomic integrity in neurons. The findings highlight the role of SUMO modifying machinery in DNA damage repair responses and transcriptional modulation in the context of neurodegenerative disease.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2021)

Article Immunology

Altered Gut Microbiota Related to Inflammatory Responses in Patients With Huntington's Disease

Gang Du et al.

Summary: This study investigated alterations in fecal microbiota and inflammatory cytokine responses in Huntington's disease (HD) patients. The results showed that HD patients had changes in microbiota diversity and structure, with some microbial components correlated with clinical characteristics and cytokine levels. The findings provide insight into the pathogenesis of HD and the potential role of gut dysbiosis in the disease.

FRONTIERS IN IMMUNOLOGY (2021)

Review Biochemistry & Molecular Biology

Involvement of the Protein Ras Homolog Enriched in the Striatum, Rhes, in Dopaminergic Neurons' Degeneration: Link to Parkinson's Disease

Marcello Serra et al.

Summary: Rhes is a gene regulated by thyroid hormones that modulates dopamine neurotransmission through inhibition of the striatal cAMP/PKA pathway. It is expressed at high levels in the dorsal striatum and is involved in the survival of midbrain dopaminergic neurons, pointing towards a modulation of autophagy and mitophagy processes.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

Review Neurosciences

Huntingtin and the Synapse

Jessica C. Barron et al.

Summary: Huntington disease is a monogenic disorder characterized by motor, psychiatric, and cognitive symptoms. While there is currently no cure, RNA-targeting therapies are being developed to reduce the production of mutant proteins. However, these therapies may inadvertently lower levels of non-pathogenic proteins, potentially leading to synaptic dysfunction.

FRONTIERS IN CELLULAR NEUROSCIENCE (2021)

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Targeting SUMO Signaling to Wrestle Cancer

Jessie S. Kroonen et al.

Summary: The SUMO signaling cascade is crucial for gene expression, genome integrity, and cell cycle progression. Small molecule inhibitors targeting SUMOylation pathway have potential as a new therapeutic strategy for cancer, leading to reduced cancer cell proliferation and increased antitumor immune response. Ongoing research is focusing on identifying tumor types that can be effectively treated with SUMOylation inhibitors.

TRENDS IN CANCER (2021)

Review Mathematical & Computational Biology

Post-translational modifications in proteins: resources, tools and prediction methods

Shahin Ramazi et al.

Summary: Posttranslational modifications (PTMs) involve modifications to amino acid side chains in proteins after biosynthesis, affecting various aspects of protein functions. Disruptions in PTMs can lead to diseases, emphasizing the need for computational methods to predict PTMs. High-throughput experimental methods for PTM discovery are laborious, prompting the exploration of computational tools and databases to advance research in this area.

DATABASE-THE JOURNAL OF BIOLOGICAL DATABASES AND CURATION (2021)

Article Geriatrics & Gerontology

SUMOylation in Neurodegenerative Diseases

Andrea Princz et al.

GERONTOLOGY (2020)

Article Biochemistry & Molecular Biology

SUMOylation Prevents Huntingtin Fibrillization and Localization onto Lipid Membranes

Faezeh Sedighi et al.

ACS CHEMICAL NEUROSCIENCE (2020)

Review Biochemistry & Molecular Biology

Molecular mechanisms in SUMO conjugation

Nathalia Varejao et al.

BIOCHEMICAL SOCIETY TRANSACTIONS (2020)

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Sumoylation on its 25th anniversary: mechanisms, pathology, and emerging concepts

Arda B. Celen et al.

FEBS JOURNAL (2020)

Article Multidisciplinary Sciences

Loss of Hap1 selectively promotes striatal degeneration in Huntington disease mice

Qiong Liu et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2020)

Review Clinical Neurology

Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities

Sarah J. Tabrizi et al.

NATURE REVIEWS NEUROLOGY (2020)

Review Physiology

SUMO: FROM BENCH TO BEDSIDE

Hui-Ming Chang et al.

PHYSIOLOGICAL REVIEWS (2020)

Review Chemistry, Multidisciplinary

Deciphering protein post-translational modifications using chemical biology tools

Anne C. Conibear

NATURE REVIEWS CHEMISTRY (2020)

Review Cell Biology

SUMO proteins in the cardiovascular system: friend or foe?

Prithviraj Manohar Vijaya Shetty et al.

JOURNAL OF BIOMEDICAL SCIENCE (2020)

Review Immunology

Inflammation in Huntington's disease: A few new twists on an old tale

Priscila Aparecida Costa Valadao et al.

JOURNAL OF NEUROIMMUNOLOGY (2020)

Review Clinical Neurology

Huntington disease: A quarter century of progress since the gene discovery

Claudia M. Testa et al.

JOURNAL OF THE NEUROLOGICAL SCIENCES (2019)

Review Neurosciences

A role for autophagy in Huntington's disease

Katherine R. Croce et al.

NEUROBIOLOGY OF DISEASE (2019)

Review Clinical Neurology

Huntington's disease: a clinical review

P. McColgan et al.

EUROPEAN JOURNAL OF NEUROLOGY (2018)

Review Pharmacology & Pharmacy

Regulation of HSF1 protein stabilization: An updated review

Chao Huang et al.

EUROPEAN JOURNAL OF PHARMACOLOGY (2018)

Article Pharmacology & Pharmacy

An overview of the possible therapeutic role of SUMOylation in the treatment of Alzheimer's disease

S. Marcelli et al.

PHARMACOLOGICAL RESEARCH (2018)

Article Clinical Neurology

Body Weight Is a Robust Predictor of Clinical Progression in Huntington Disease

Jorien M. M. van der Burg et al.

ANNALS OF NEUROLOGY (2017)

Review Neurosciences

Neuropsychiatric Burden in Huntington's Disease

Ricardo Augusto Paoli et al.

BRAIN SCIENCES (2017)

Article Biochemistry & Molecular Biology

Battling Alzheimer's Disease: Targeting SUMOylation-Mediated Pathways

Wagner Carbolin Martins et al.

NEUROCHEMICAL RESEARCH (2016)

Review Biochemistry & Molecular Biology

SUMO-regulated mitochondrial function in Parkinson's disease

Ana Cristina Guerra de Souza et al.

JOURNAL OF NEUROCHEMISTRY (2016)

Review Biochemistry & Molecular Biology

Cellular maintenance of nuclear protein homeostasis

Pamela S. Gallagher et al.

CELLULAR AND MOLECULAR LIFE SCIENCES (2014)

Review Oncology

The role of Rhes, Ras homolog enriched in striatum, in neurodegenerative processes

Laura M. Harrison et al.

EXPERIMENTAL CELL RESEARCH (2013)

Article Neurosciences

SUMO-1 is Associated with a Subset of Lysosomes in Glial Protein Aggregate Diseases

Mathew B. Wong et al.

NEUROTOXICITY RESEARCH (2013)

Article Cell Biology

SUMO-2 and PIAS1 Modulate Insoluble Mutant Huntingtin Protein Accumulation

Jacqueline Gire O'Rourke et al.

CELL REPORTS (2013)

Review Neurosciences

Biological markers of cognition in prodromal Huntington's disease: A review

Kathryn V. Papp et al.

BRAIN AND COGNITION (2011)

Review Neurosciences

Dynamin-related protein 1 and mitochondrial fragmentation in neurodegenerative diseases

P. Hemachandra Reddy et al.

BRAIN RESEARCH REVIEWS (2011)

Review Cell Biology

The regulation of mitochondrial morphology: Intricate mechanisms and dynamic machinery

Catherine S. Palmer et al.

CELLULAR SIGNALLING (2011)

Article Cell Biology

Does Huntingtin play a role in selective macroautophagy?

Joan S. Steffan

CELL CYCLE (2010)

Article Biochemistry & Molecular Biology

Rhes, a Physiologic Regulator of Sumoylation, Enhances Cross-sumoylation between the Basic Sumoylation Enzymes E1 and Ubc9

Srinivasa Subramaniam et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2010)

Article Cell Biology

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

Leslie Michels Thompson et al.

JOURNAL OF CELL BIOLOGY (2009)

Article Cell Biology

SUMOylation and deSUMOylation at a glance

Yonggang Wang et al.

JOURNAL OF CELL SCIENCE (2009)

Article Clinical Neurology

Beyond the brain: widespread pathology in Huntington's disease

Jorien M. M. van der Burg et al.

LANCET NEUROLOGY (2009)

Article Clinical Neurology

Huntington disease: pathogenesis, biomarkers, and approaches to experimental therapeutics

Christopher A. Ross et al.

PARKINSONISM & RELATED DISORDERS (2009)

Article Multidisciplinary Sciences

Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity

Srinivasa Subramaniam et al.

SCIENCE (2009)

Review Biochemistry & Molecular Biology

SUMO on the road to neurodegeneration

Veronique Dorval et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2007)

Article Biochemistry & Molecular Biology

Human cystathionine β-synthase is a target for sumoylation

Omer Kabil et al.

BIOCHEMISTRY (2006)

Article Multidisciplinary Sciences

SUMO modification of Huntingtin and Huntington's disease pathology

JS Steffan et al.

SCIENCE (2004)

Article Neurosciences

SUMO-1 marks the nuclear inclusions in familial neuronal intranuclear inclusion disease

DL Pountney et al.

EXPERIMENTAL NEUROLOGY (2003)

Article Biochemistry & Molecular Biology

Enhanced SUMOylation in polyglutamine diseases

H Ueda et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2002)