Cholesterol metabolism in Huntington disease

Cholesterol metabolism in Huntington disease - PubMed

The CNS is rich in cholesterol, which is essential for neuronal development and survival, synapse maturation, and optimal synaptic activity.

Cholesterol metabolism in Huntington disease - Nature

Research has shown that Huntington disease, a progressive and fatal neurodegenerative disorder resulting from polyglutamine expansion in the ...

Altered Cholesterol Homeostasis in Huntington's Disease - PMC

The whole body cholesterol metabolism seems to be altered in HD. Cholesterol metabolism changes may also results of dysfunction related to food digestion and ...

The impairment of cholesterol metabolism in Huntington disease

The impairment of the cholesterol metabolism was found to be proportional to the CAG repeat length and to the load of mutant HTT.

Dysregulated Brain Cholesterol Metabolism Is Linked to ...

Huntington's disease (HD) is an autosomal-dominant, neurodegenerative disorder caused by a CAG repeat expansion in exon-1 of the huntingtin (HTT) ...

CYP46A1 gene therapy deciphers the role of brain cholesterol ...

Recently, disturbances of cholesterol homeostasis have been discovered in patients and Huntington's disease mouse models (Karasinska and Hayden, ...

Investigating the Effects of Brain Cholesterol Metabolism Using ...

HD is a progressive, genetic neurodegenerative disorder linked to cholesterol dysregulation and subsequent protein aggregation in the brain.

Study of cholesterol metabolism in Huntington′s disease

There is a lower synthesis and transport of cholesterol from astrocytes to neurons via ApoE. In primary oligodendrocytes, mutant huntingtin inhibits the ...

Evidence for altered cholesterol metabolism in Huntington's disease ...

Alteration of brain cholesterol synthesis and metabolism has been demonstrated in several Huntington's disease (HD) mouse and cell models ...

Development of clinical Gene therapy for Huntington's disease | ANR

Increasing evidences suggest that dysfunction of brain cholesterol metabolism plays a major role in neurodegenerative diseases particularly in Huntington ...

Altered lipid metabolism in Drosophila model of Huntington's disease

We document that diseased flies exhibit change in weight due to an altered lipid metabolism, as evident from considerably high lipid levels at ...

Evidence for altered cholesterol metabolism in Huntington's disease ...

Alteration of brain cholesterol synthesis and metabolism has been demonstrated in several. Huntington's disease (HD) mouse and cell models; however, less is ...

Altered cholesterol and fatty acid metabolism in Huntington disease

Huntington disease is also associated with a pattern of insulin resistance characterized by a catabolic state, resulting in weight loss and a lower body mass ...

Cholesterol Metabolism in the Brain and Its Association with ...

Abnormal cholesterol metabolism in the brain is also associated with many neurodegenerative disorders, such as AD, PD, Huntington's disease (HD) ...

Dysfunction of the Cholesterol Biosynthetic Pathway in Huntington's ...

The expansion of a polyglutamine tract in the ubiquitously expressed huntingtin protein causes Huntington's disease (HD), a dominantly inherited ...

CYP46A1, the rate-limiting enzyme for cholesterol degradation, is ...

Dysregulation of cholesterol synthesis is implicated in Huntington's disease. Boussicault et al. show that expression of CYP46A1, ...

The impairment of cholesterol metabolism in Huntington disease.

98 Citations · Altered Cholesterol Homeostasis in Huntington's Disease · Dysregulated Brain Cholesterol Metabolism Is Linked to Neuroinflammation in ...

Huntington's Disease Therapies May Target Cholesterol Metabolism...

Researchers have shown that manipulation of cholesterol levels in striatal neurons can improve motor deficits in an animal model of Huntington's.

Evidence for altered cholesterol metabolism in Huntington's disease ...

Aims Cholesterol plays an essential role in membrane structure and function, being especially important in the brain. Alteration of brain cholesterol ...

Role of Cyp46 in cholesterol metabolism and neuroprotection ... - ANR

Huntington's Disease (HD) is a genetic neurodegenerative disease, due to an unstable expansion of the CAG repeat sequence, located in exon 1 of the Htt gene ...