A series of N-terminal epitope tagged Hdh knock-in alleles expressing normal and mutant huntingtin: their application to understanding the effect of increasing the length of normal huntingtin’s polyglutamine stretch on CAG140 mouse model pathogenesis

Native Mutant Huntingtin in Human Brain

(2012) Ellen Sapp et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion

(2012) J.- M. Lee et al.   NEUROLOGY

Ciliogenesis is regulated by a huntingtin-HAP1-PCM1 pathway and is altered in Huntington disease

(2011) Guy Keryer et al.   JOURNAL OF CLINICAL INVESTIGATION

The composition of the polyglutamine-containing proteins influences their co-aggregation properties

(2010) Daniel Bąk et al.   CELL BIOLOGY INTERNATIONAL

Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

(2010) Christian Landles et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

(2010) J. Miller et al.   JOURNAL OF NEUROSCIENCE

The number of CAG repeats within the normal allele does not influence the age of onset in Huntington's disease

(2010) Jiří Klempíř et al.   MOVEMENT DISORDERS

Huntingtin Is Required for Mitotic Spindle Orientation and Mammalian Neurogenesis

(2010) Juliette D. Godin et al.   NEURON

Modulation of Mutant Huntingtin N-Terminal Cleavage and Its Effect on Aggregation and Cell Death

(2010) Katrin Juenemann et al.   NEUROTOXICITY RESEARCH

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

Deletion of the Huntingtin Polyglutamine Stretch Enhances Neuronal Autophagy and Longevity in Mice

(2010) Shuqiu Zheng et al.   PLoS Genetics

Huntingtin facilitates polycomb repressive complex 2

(2009) Ihn Sik Seong et al.   HUMAN MOLECULAR GENETICS

Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease

(2009) N. A. Aziz et al.   NEUROLOGY

Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease

(2008) Chuan-En Wang et al.   HUMAN MOLECULAR GENETICS

CAG repeat lengths ≥335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse

(2008) I. Dragatsis et al.   NEUROBIOLOGY OF DISEASE

Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

(2008) M.A. Hickey et al.   NEUROSCIENCE