Juvenile Huntington's Disease Skin Fibroblasts Respond with Elevated Parkin Level and Increased Proteasome Activity as a Potential Mechanism to Counterbalance the Pathological Consequences of Mutant Huntingtin Protein. [PDF]
Int J Mol Sci, 2019 Huntington’s disease (HD) is an inherited neurodegenerative disorder, caused by an abnormal polyglutamine (polyQ) expansion in the huntingtin protein (Htt).
Aladdin A +4 more
europepmc +4 more sources
Current Pharmacological Management in Juvenile Huntington's Disease. [PDF]
PLoS Curr, 2012 Background: The clinical presentation of Juvenile Huntington’s Disease (JHD) can be very different from adult-onset HD with little evidence to guide symptomatic management. Aim: To survey the current use of pharmacological treatments for JHD.
Robertson L +7 more
europepmc +3 more sources
Neuropathological Comparison of Adult Onset and Juvenile Huntington's Disease with Cerebellar Atrophy: A Report of a Father and Son. [PDF]
J Huntingtons Dis, 2017 Latimer CS +9 more
europepmc +4 more sources
Abnormal Weight and Body Mass Index in Children with Juvenile Huntington's Disease. [PDF]
J Huntingtons Dis, 2015 Tereshchenko A +6 more
europepmc +4 more sources
The personal experience of parenting a child with Juvenile Huntington’s Disease: perceptions across Europe [PDF]
European Journal of Human Genetics, 2013 The study reported here presents a detailed description of what it is like to parent a child with juvenile Huntington’s disease in families across four European countries. Its primary aim was to develop and extend findings from a previous UK study.
V. Eatough +14 more
semanticscholar +4 more sources
Special Issue: Juvenile Onset Huntington’s Disease [PDF]
Brain Sciences, 2020 [...]
Peg C. Nopoulos
doaj +2 more sources
Behavioral Deficits in Juvenile Onset Huntington’s Disease [PDF]
Brain Sciences, 2020 Reports of behavioral disturbance in Juvenile-Onset Huntington’s Disease (JOHD) have been based primarily on qualitative caregiver reports or retrospective medical record reviews.
Kathleen E. Langbehn +7 more
doaj +2 more sources
Autonomic Changes in Juvenile-Onset Huntington’s Disease [PDF]
Brain Sciences, 2020 Patients with adult-onset Huntington’s Disease (AOHD) have been found to have dysfunction of the autonomic nervous system that is thought to be secondary to neurodegeneration causing dysfunction of the brain–heart axis. However, this relationship has not
Jordan L. Schultz, Peg C. Nopoulos
doaj +2 more sources
Brain microvascular endothelial cell dysfunction in an isogenic juvenile iPSC model of Huntington’s disease [PDF]
Fluids and Barriers of the CNS, 2022 Huntington’s disease (HD) is an inherited neurodegenerative disease caused by expansion of cytosine–adenine–guanine (CAG) repeats in the huntingtin gene, which leads to neuronal loss and decline in cognitive and motor function.
Raleigh M. Linville +5 more
doaj +2 more sources
The Association between CAG Repeat Length and Age of Onset of Juvenile-Onset Huntington’s Disease [PDF]
Brain Sciences, 2020 There is a known negative association between cytosine–adenine–guanine (CAG) repeat length and the age of motor onset (AMO) in adult-onset Huntington’s Disease (AOHD).
Jordan L. Schultz +2 more
doaj +2 more sources