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Search results 901 to 1000 out of 1119 for Htt

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Hits by Strain

Type Details Score
Strain
Attribute String: coisogenic, mutant strain, transgenic
Allele
Name: transgene insertion 52, Christopher A Ross
Allele Type: Transgenic
Attribute String: Humanized sequence, Inserted expressed sequence
Allele
Name: transgene insertion N, X William Yang
Allele Type: Transgenic
Attribute String: Humanized sequence, Inserted expressed sequence
Allele
Name: transgene insertion L, X William Yang
Allele Type: Transgenic
Attribute String: Humanized sequence, Inserted expressed sequence
Allele
Name: transgene insertion A, X William Yang
Allele Type: Transgenic
Attribute String: Humanized sequence, Inserted expressed sequence
Strain
Attribute String: congenic, mutant strain, targeted mutation
Genotype
Symbol: Hm/Hm<+> Del(5D5Mit73-D5Mit351)5Jcs/?
Background: involves: 129S4/SvJae * C57BL/6J
Zygosity: cx
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt
Background: involves: 129S1/Sv * 129X1/SvJ * Swiss Webster
Zygosity: ht
Has Mutant Allele: true
Genotype
Symbol: Tg(HTT*97Q)LXwy/?
Background: FVB-Tg(HTT*97Q)LXwy
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Tg(UBC-GFP/HTT*84Q)22Shya/?
Background: involves: FVB
Zygosity: ot
Has Mutant Allele: true
Protein
Organism: Mus musculus/domesticus
Length: 3119  
Fragment?: false
Protein
Organism: Mus musculus/domesticus
Length: 3120  
Fragment?: false
Publication
First Author: Dickey AS
Year: 2016
Journal: Nat Med
Title: PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically.
Volume: 22
Issue: 1
Pages: 37-45
Publication
First Author: Caron NS
Year: 2021
Journal: J Neurosci
Title: Mutant Huntingtin Is Cleared from the Brain via Active Mechanisms in Huntington Disease.
Volume: 41
Issue: 4
Pages: 780-796
Publication
First Author: Clark RM
Year: 2001
Journal: Genetics
Title: Reciprocal mouse and human limb phenotypes caused by gain- and loss-of-function mutations affecting Lmbr1.
Volume: 159
Issue: 2
Pages: 715-26
Publication
First Author: Valenza M
Year: 2007
Journal: Hum Mol Genet
Title: Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation.
Volume: 16
Issue: 18
Pages: 2187-98
Allele
Name: transgene insertion I, X William Yang
Allele Type: Transgenic
Attribute String: Conditional ready, Humanized sequence, Inserted expressed sequence
Publication
First Author: Tang TS
Year: 2005
Journal: Proc Natl Acad Sci U S A
Title: Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
Volume: 102
Issue: 7
Pages: 2602-7
Strain
Attribute String: coisogenic, mutant strain, transgenic
Strain
Attribute String: transgenic, mutant strain, coisogenic
Strain
Attribute String: coisogenic, mutant strain, transgenic
Strain
Attribute String: transgenic, mutant strain, coisogenic
Strain
Attribute String: transgenic, mutant strain, coisogenic
Strain
Attribute String: congenic, endonuclease-mediated mutation, mutant strain
Genotype
Symbol: Lmbr1/Lmbr1<+> Del(5D5Mit73-D5Mit351)5Jcs/?
Background: mixed
Zygosity: cx
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt
Background: involves: 129S1/Sv * 129X1/SvJ * Swiss Webster
Zygosity: ht
Has Mutant Allele: true
Genotype
Symbol: Tg(HTT*)BXwy/?
Background: FVB-Tg(HTT*)BXwy
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Tg(HTT*)1Xwy/?
Background: FVB-Tg(HTT*)1Xwy
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt Tg(YAC18)18Hay/?
Background: involves: FVB/N
Zygosity: cx
Has Mutant Allele: true
Genotype
Symbol: Tg(HTT*)NXwy/?
Background: involves: FVB/NJ
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Tg(HTT*)LXwy/?
Background: involves: FVB/NJ
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Tg(HTT*)AXwy/?
Background: involves: FVB/NJ
Zygosity: ot
Has Mutant Allele: true
Publication
First Author: Roberts SL
Year: 2020
Journal: Hum Mol Genet
Title: Bim contributes to the progression of Huntington's disease-associated phenotypes.
Volume: 29
Issue: 2
Pages: 216-227
Publication
First Author: Holmes A
Year: 2003
Journal: Biol Psychiatry
Title: Abnormal behavioral phenotypes of serotonin transporter knockout mice: parallels with human anxiety and depression.
Volume: 54
Issue: 10
Pages: 953-9
Publication
First Author: Singaraja RR
Year: 2011
Journal: Hum Mol Genet
Title: Altered palmitoylation and neuropathological deficits in mice lacking HIP14.
Volume: 20
Issue: 20
Pages: 3899-909
Publication
First Author: Zhu J
Year: 2020
Journal: Mol Cell
Title: GIT/PIX Condensates Are Modular and Ideal for Distinct Compartmentalized Cell Signaling.
Volume: 79
Issue: 5
Pages: 782-796.e6
Publication
First Author: Bagrodia S
Year: 1999
Journal: Trends Cell Biol
Title: Pak to the future.
Volume: 9
Issue: 9
Pages: 350-5
Publication
First Author: Flanders JA
Year: 2003
Journal: FEBS Lett
Title: The Cbl proteins are binding partners for the Cool/Pix family of p21-activated kinase-binding proteins.
Volume: 550
Issue: 1-3
Pages: 119-23
Publication
First Author: Feng Q
Year: 2002
Journal: J Biol Chem
Title: Regulation of the Cool/Pix proteins: key binding partners of the Cdc42/Rac targets, the p21-activated kinases.
Volume: 277
Issue: 7
Pages: 5644-50
Publication
First Author: Eriguchi M
Year: 2010
Journal: J Neurol Sci
Title: alpha Pix enhances mutant huntingtin aggregation.
Volume: 290
Issue: 1-2
Pages: 80-5
Protein Domain
Type: Domain
Description: There are two forms of Pix proteins: alpha Pix (also called Rho guanine nucleotide exchange factor (GEF) 6, 90Cool-2 or ARHGEF6) and beta Pix (GEF7, p85Cool-1 or ARHGEF7), which activate small GTPases by exchanging bound GDP for free GTP. betaPix contains an N-terminal SH3 domain, a RhoGEF/DH domain, a PH domain, a GIT1 binding domain (GBD), and a C-terminal coiled-coil (CC) domain []. It acts as a GEF for both Cdc42 and Rac1 [], and plays important roles in regulating neuroendocrine exocytosis, focal adhesion maturation, cell migration, synaptic vesicle localization, and insulin secretion [, , , ]. alphaPix differs in that it contains a calponin homology (CH) domain, which interacts with beta-parvin, N-terminal to the SH3 domain. alphaPix is an exchange factor for Rac1 and Cdc42 and mediates Pak activation on cell adhesion to fibronectin. Mutations in alphaPix can cause X-linked mental retardation. alphaPix also interacts with Huntington's disease protein (htt), and enhances the aggregation of mutant htt (muthtt) by facilitating SDS-soluble muthtt-muthtt interactions. The DH-PH domain of a Pix was required for its binding to htt. In the majority of Rho GEF proteins, the DH-PH domain is responsible for the exchange activity [, , , , ].This entry represents the PH domain of ARHGEF6 and ARHGEF7.
HT Experiment  
Experiment Type: transcription profiling by array
Study Type: Baseline
Source: GEO
Publication
First Author: Shehadeh J
Year: 2006
Journal: Neurobiol Dis
Title: Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease.
Volume: 21
Issue: 2
Pages: 392-403
Strain
Attribute String: coisogenic, transgenic
Strain
Attribute String: transgenic, coisogenic
Publication
First Author: Doria JG
Year: 2018
Journal: J Neurochem
Title: The mGluR5 positive allosteric modulator VU0409551 improves synaptic plasticity and memory of a mouse model of Huntington's disease.
Volume: 147
Issue: 2
Pages: 222-239
Publication  
First Author: Abada YS
Year: 2013
Journal: Behav Brain Res
Title: Motor, emotional and cognitive deficits in adult BACHD mice: a model for Huntington's disease.
Volume: 238
Pages: 243-51
Publication
First Author: Aharony I
Year: 2015
Journal: Hum Mol Genet
Title: A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits.
Volume: 24
Issue: 9
Pages: 2604-14
Publication  
First Author: Cheng S
Year: 2018
Journal: Exp Neurol
Title: Therapeutic efficacy of regulable GDNF expression for Huntington's and Parkinson's disease by a high-induction, background-free "GeneSwitch" vector.
Volume: 309
Pages: 79-90
Publication  
First Author: Dickey AS
Year: 2017
Journal: Sci Transl Med
Title: PPARδ activation by bexarotene promotes neuroprotection by restoring bioenergetic and quality control homeostasis.
Volume: 9
Issue: 419
Publication  
First Author: Olmo IG
Year: 2021
Journal: ASN Neuro
Title: High-Throughput Sequencing of BACHD Mice Reveals Upregulation of Neuroprotective miRNAs at the Pre-Symptomatic Stage of Huntington's Disease.
Volume: 13
Pages: 17590914211009857
Publication
First Author: Kuljis DA
Year: 2016
Journal: PLoS One
Title: Sex Differences in Circadian Dysfunction in the BACHD Mouse Model of Huntington's Disease.
Volume: 11
Issue: 2
Pages: e0147583
Publication
First Author: Mantovani S
Year: 2016
Journal: Hum Mol Genet
Title: Motor deficits associated with Huntington's disease occur in the absence of striatal degeneration in BACHD transgenic mice.
Volume: 25
Issue: 9
Pages: 1780-91
Publication  
First Author: Diaz Escarcega R
Year: 2024
Journal: Mol Cell Neurosci
Title: Sphingosine kinase 2 regulates protein ubiquitination networks in neurons.
Volume: 130
Pages: 103948
Publication
First Author: Conroy F
Year: 2022
Journal: Nat Commun
Title: Chemical engineering of therapeutic siRNAs for allele-specific gene silencing in Huntington's disease models.
Volume: 13
Issue: 1
Pages: 5802
Publication
First Author: Le Cann K
Year: 2021
Journal: Sci Rep
Title: The difficulty to model Huntington's disease in vitro using striatal medium spiny neurons differentiated from human induced pluripotent stem cells.
Volume: 11
Issue: 1
Pages: 6934
Publication  
First Author: Lee W
Year: 2013
Journal: Neurobiol Dis
Title: Enhanced Ca(2+)-dependent glutamate release from astrocytes of the BACHD Huntington's disease mouse model.
Volume: 58
Pages: 192-9
Publication  
First Author: Rocher AB
Year: 2016
Journal: Neurobiol Dis
Title: Synaptic scaling up in medium spiny neurons of aged BACHD mice: A slow-progression model of Huntington's disease.
Volume: 86
Pages: 131-9
Publication
First Author: Hult Lundh S
Year: 2013
Journal: Hum Mol Genet
Title: Hypothalamic expression of mutant huntingtin contributes to the development of depressive-like behavior in the BAC transgenic mouse model of Huntington's disease.
Volume: 22
Issue: 17
Pages: 3485-97
Publication
First Author: Mattis VB
Year: 2015
Journal: Hum Mol Genet
Title: HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity.
Volume: 24
Issue: 11
Pages: 3257-71
Publication
First Author: Schroeder AM
Year: 2016
Journal: PLoS One
Title: Cardiac Dysfunction in the BACHD Mouse Model of Huntington's Disease.
Volume: 11
Issue: 1
Pages: e0147269
Publication  
First Author: Radulescu CI
Year: 2019
Journal: Neurobiol Dis
Title: Manipulation of microbiota reveals altered callosal myelination and white matter plasticity in a model of Huntington disease.
Volume: 127
Pages: 65-75
Publication  
First Author: Gosset P
Year: 2020
Journal: Neurobiol Dis
Title: Evidence for the spread of human-derived mutant huntingtin protein in mice and non-human primates.
Volume: 141
Pages: 104941
Publication  
First Author: Duarte F
Year: 2023
Journal: Mol Ther Methods Clin Dev
Title: Semi-automated workflows to quantify AAV transduction in various brain areas and predict gene editing outcome for neurological disorders.
Volume: 29
Pages: 254-270
Strain
Attribute String: congenic, endonuclease-mediated mutation, mutant strain, targeted mutation
Genotype
Symbol: Tg(HTT*97Q)IXwy/?
Background: FVB-Tg(HTT*97Q)IXwy
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt Tg(HTT*)1Xwy/?
Background: involves: 129S/SvEv * FVB
Zygosity: cx
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt Tg(HTT*)BXwy/?
Background: involves: 129S/SvEv * FVB
Zygosity: cx
Has Mutant Allele: true
Genotype
Symbol: Tg(Prnp-HTT*82Q)52Caro/?
Background: involves: C3H * C57BL/6
Zygosity: ot
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt Tg(CAG-cre/Esr1*)5Amc/?
Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * Swiss Webster
Zygosity: cn
Has Mutant Allele: true
Genotype
Symbol: Htt/Htt<+> Tyrobp/Tyrobp
Background: involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6
Zygosity: cx
Has Mutant Allele: true
Publication  
First Author: Baird D
Year: 2006
Journal: Methods Enzymol
Title: Biochemical characterization of the Cool (Cloned-out-of-Library)/Pix (Pak-interactive exchange factor) proteins.
Volume: 406
Pages: 58-69
Publication
First Author: Kim SH
Year: 2016
Journal: Hum Mol Genet
Title: Allele-specific regulation of mutant Huntingtin by Wig1, a downstream target of p53.
Volume: 25
Issue: 12
Pages: 2514-2524
Publication
First Author: Soylu-Kucharz R
Year: 2022
Journal: iScience
Title: IKKβ signaling mediates metabolic changes in the hypothalamus of a Huntington disease mouse model.
Volume: 25
Issue: 2
Pages: 103771
Publication
First Author: Ichikawa M
Year: 2008
Journal: Biochem Biophys Res Commun
Title: Expression analysis for inverted effects of serotonin transporter inactivation.
Volume: 368
Issue: 1
Pages: 43-9
Publication
First Author: Powell JE
Year: 2022
Journal: Sci Adv
Title: Targeted gene silencing in the nervous system with CRISPR-Cas13.
Volume: 8
Issue: 3
Pages: eabk2485
Publication
First Author: Fernández-Nogales M
Year: 2014
Journal: Nat Med
Title: Huntington's disease is a four-repeat tauopathy with tau nuclear rods.
Volume: 20
Issue: 8
Pages: 881-5
Publication  
First Author: Liu SY
Year: 2018
Journal: Brain Res
Title: Intravenous immunoglobulin ameliorates motor and cognitive deficits and neuropathology in R6/2 mouse model of Huntington's disease by decreasing mutant huntingtin protein level and normalizing NF-κB signaling pathway.
Volume: 1697
Pages: 21-33
Publication
First Author: Lin JT
Year: 2013
Journal: Mol Cell Biol
Title: Regulation of feedback between protein kinase A and the proteasome system worsens Huntington's disease.
Volume: 33
Issue: 5
Pages: 1073-84
Publication
First Author: Dargaei Z
Year: 2018
Journal: Proc Natl Acad Sci U S A
Title: Restoring GABAergic inhibition rescues memory deficits in a Huntington's disease mouse model.
Volume: 115
Issue: 7
Pages: E1618-E1626
Publication
First Author: Nakamori M
Year: 2020
Journal: Nat Genet
Title: A slipped-CAG DNA-binding small molecule induces trinucleotide-repeat contractions in vivo.
Volume: 52
Issue: 2
Pages: 146-159
Publication
First Author: Li M
Year: 2009
Journal: Neurobiol Dis
Title: Y-27632 improves rotarod performance and reduces huntingtin levels in R6/2 mice.
Volume: 36
Issue: 3
Pages: 413-20
Publication
First Author: Ochaba J
Year: 2016
Journal: Neuron
Title: PIAS1 Regulates Mutant Huntingtin Accumulation and Huntington's Disease-Associated Phenotypes In Vivo.
Volume: 90
Issue: 3
Pages: 507-20
Publication
First Author: Ferlazzo GM
Year: 2023
Journal: Nat Commun
Title: Genome-wide screening in pluripotent cells identifies Mtf1 as a suppressor of mutant huntingtin toxicity.
Volume: 14
Issue: 1
Pages: 3962
Publication
First Author: Miller JP
Year: 2010
Journal: Neuron
Title: Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease.
Volume: 67
Issue: 2
Pages: 199-212
Publication
First Author: Crittenden JR
Year: 2010
Journal: Hum Mol Genet
Title: CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease.
Volume: 19
Issue: 9
Pages: 1756-65
Publication
First Author: Miller TW
Year: 2005
Journal: Neurobiol Dis
Title: A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease.
Volume: 19
Issue: 1-2
Pages: 47-56
Publication
First Author: Yano H
Year: 2014
Journal: Nat Neurosci
Title: Inhibition of mitochondrial protein import by mutant huntingtin.
Volume: 17
Issue: 6
Pages: 822-31
Publication
First Author: Luo S
Year: 2005
Journal: J Cell Biol
Title: Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity.
Volume: 169
Issue: 4
Pages: 647-56
Publication
First Author: Tsunemi T
Year: 2012
Journal: Sci Transl Med
Title: PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function.
Volume: 4
Issue: 142
Pages: 142ra97
Publication
First Author: Van Raamsdonk JM
Year: 2007
Journal: Neurobiol Dis
Title: Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain.
Volume: 26
Issue: 1
Pages: 189-200
Publication
First Author: Haenig C
Year: 2020
Journal: Cell Rep
Title: Interactome Mapping Provides a Network of Neurodegenerative Disease Proteins and Uncovers Widespread Protein Aggregation in Affected Brains.
Volume: 32
Issue: 7
Pages: 108050
Publication
First Author: Klyczek KK
Year: 1984
Journal: J Exp Med
Title: T cell surface I-J glycoprotein. Concerted action of chromosome-4 and -17 genes forms an epitope dependent on alpha-D-mannosyl residues.
Volume: 159
Issue: 6
Pages: 1604-17
Publication
First Author: Bengel D
Year: 1997
Journal: Brain Res
Title: Cellular localization and expression of the serotonin transporter in mouse brain.
Volume: 778
Issue: 2
Pages: 338-45
Publication
First Author: Ehrnhoefer DE
Year: 2009
Journal: Dis Model Mech
Title: Mouse models of Huntington disease: variations on a theme.
Volume: 2
Issue: 3-4
Pages: 123-9
Publication  
First Author: Sancho M
Year: 2014
Journal: Neuroscience
Title: Inactivation of Apaf1 reduces the formation of mutant huntingtin-dependent aggregates and cell death.
Volume: 262
Pages: 83-91
Publication
First Author: Momboisse F
Year: 2010
Journal: Cell Mol Neurobiol
Title: The Rho guanine nucleotide exchange factors Intersectin 1L and β-Pix control calcium-regulated exocytosis in neuroendocrine PC12 cells.
Volume: 30
Issue: 8
Pages: 1327-33
Publication
First Author: Legg K
Year: 2011
Journal: Nat Rev Mol Cell Biol
Title: Cell migration: keeping young and mobile with β-PIX.
Volume: 12
Issue: 5
Pages: 278
Publication
First Author: Sun Y
Year: 2011
Journal: J Neurosci
Title: β-Pix modulates actin-mediated recruitment of synaptic vesicles to synapses.
Volume: 31
Issue: 47
Pages: 17123-33
Publication
First Author: Koh SH
Year: 2012
Journal: Stem Cells Dev
Title: β-PIX is critical for transplanted mesenchymal stromal cell migration.
Volume: 21
Issue: 11
Pages: 1989-99