Beyond the brain: widespread pathology in Huntington's disease (Q33483188)

27 July 2017

title

Beyond the brain: widespread pathology in Huntington's disease (English)

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27 July 2017

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3

Patrik Brundin

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ORCID Public Data File 2021

27 July 2017

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author name string

Jorien M M van der Burg

series ordinal

1

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27 July 2017

Maria Björkqvist

series ordinal

2

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27 July 2017

publication date

1 August 2009

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27 July 2017

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27 July 2017

volume

8

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27 July 2017

issue

8

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27 July 2017

page(s)

765-774

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

27 July 2017

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7 January 2021

Characterization and localization of the Huntington disease gene product

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Huntington's disease gene (IT15) is widely expressed in human and rat tissues

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Normal huntingtin function: an alternative approach to Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Huntingtin-deficient zebrafish exhibit defects in iron utilization and development.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Correction of hemophilia B in canine and murine models using recombinant adeno-associated viral vectors

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Genotype-dependent priming to self- and xeno-cannibalism in heterozygous and homozygous lymphoblasts from patients with Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Evidence of apoptosis and mitochondrial abnormalities in peripheral blood cells of Huntington's disease patients.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Ca2+-induced permeability transition in human lymphoblastoid cell mitochondria from normal and Huntington's disease individuals.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Gene expression in Huntington's disease skeletal muscle: a potential biomarker

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Chromosomal profiles of gene expression in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Impaired PGC-1alpha function in muscle in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Formation of polyglutamine inclusions in non-CNS tissue

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Inclusion formation in Huntington's disease R6/2 mouse muscle cultures

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Protein misfolding and cardiac disease: establishing cause and effect

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Cardiomyocyte expression of a polyglutamine preamyloid oligomer causes heart failure.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

An anthropometric assessment of Huntington's disease patients and families

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Anthropometric discrimination among affected, at-risk, and not-at-risk individuals in families with Huntington disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Weight loss in early stage of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Assessment of the nutrition status of patients with Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Use of oral nutritional supplements in patients with Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Weight changes in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Early energy deficit in Huntington disease: identification of a plasma biomarker traceable during disease progression

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Weight loss in Huntington disease increases with higher CAG repeat number

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Nutritional evaluation of Huntington disease patients

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Increased metabolism in the R6/2 mouse model of Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

The metabolic profile of early Huntington's disease--a combined human and transgenic mouse study

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Factors associated with slow progression in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Increased thirst and drinking in Huntington's disease and the R6/2 mouse

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Increased frequency of diabetes mellitus in patients with Huntington's chorea

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Abnormal Glucose Tolerance and Arginine Tolerance Tests in Huntington’s Disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

The R6/2 transgenic mouse model of Huntington's disease develops diabetes due to deficient beta-cell mass and exocytosis

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Impaired glucose tolerance in the R6/1 transgenic mouse model of Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Glucose homeostasis in Huntington disease: abnormalities in insulin sensitivity and early-phase insulin secretion

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Atypical diabetes associated with inclusion formation in the R6/2 mouse model of Huntington's disease is not improved by treatment with hypoglycaemic agents.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Islet beta-cell area and hormone expression are unaltered in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Abnormalities in the functioning of adipocytes from R6/2 mice that are transgenic for the Huntington's disease mutation

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Mitochondria take center stage in aging and neurodegeneration

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Complex I defect in muscle from patients with Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington's disease subjects

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Clinical correlates of mitochondrial function in Huntington's disease muscle

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Conditions associated with Huntington's disease at death. A case-control study

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Cardiovagal modulation upon postural change is altered in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Cardiac dysfunction in the R6/2 mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Plasma testosterone in male patients with Huntington's disease: relations to severity of illness and dementia

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Testicular degeneration in Huntington disease.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Neuroendocrine disturbances in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Increased hypothalamic-pituitary-adrenal axis activity in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Aberrant A2A receptor function in peripheral blood cells in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Biological abnormalities of peripheral A(2A) receptors in a large representation of polyglutamine disorders and Huntington's disease stages

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Platelet monoamine oxidase activity in patients with Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Inflammatory cytokines inhibit myogenic differentiation through activation of nuclear factor-kappaB.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Through thick and thin: wasting, obesity, and TNF alpha

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Weight loss in neurodegenerative disorders

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Hypothalamic-endocrine aspects in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Hypothalamic dysfunction and neuroendocrine and metabolic alterations in Huntington's disease: clinical consequences and therapeutic implications.

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https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Orexin loss in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Decreased expression of hypothalamic neuropeptides in Huntington disease transgenic mice with expanded polyglutamine-EGFP fluorescent aggregates.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Exendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

DNA instability in replicating Huntington's disease lymphoblasts

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Progressive dysfunction of the cholesterol biosynthesis pathway in the R6/2 mouse model of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Metformin therapy in a transgenic mouse model of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

The glucagon-like peptides: a double-edged therapeutic sword?

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Sex hormones and brain aging

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Predict-HD and the future of therapeutic trials

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1474-4422%2809%2970178-4

7 January 2021

10.1016/S1474-4422(09)70178-4

Identifiers

DOI

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27 July 2017

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