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Calpain activation is involved in early caspase-independent neurodegeneration in the hippocampus following status epilepticus

Araújo IM, Gil J, Carreira BP, Mohapel P, Petersén Å, Pinheiro PS, Soulet D, Bahr BA, Brundin P and Carvalho CM.Journal of Neurochemistry 105: 666-676 (2008)AbstractEvidence for increased calpain activity has been described in the hippocampus of rodent models of temporal lobe epilepsy. However, it is not known whether calpains are involved in the cell death that accompanies seizures. In this work,

https://www.huntington-research.lu.se/calpain-activation-involved-early-caspase-independent-neurodegeneration-hippocampus-following-status - 2025-04-29

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

van der Burg JMM, Bacos K, Wood N, Lindqvist A, Wierup N, Wamsteeker J, Deierborg-Olsson T, Mulder H, Erlanson-Albertsson C, Morton J, Brundin P, Björkqvist M* and Petersén Å*.Neurobiology of Disease 29: 41-51 (2008) *equal contribution AbstractHuntington's disease (HD) is a hereditary disorder characterized by personality changes, chorea, dementia and weight loss. The cause of this weight loss is

https://www.huntington-research.lu.se/increased-metabolism-r62-mouse-model-huntingtons-disease - 2025-04-29

Proteomic profiling of plasma in Hungtinton's disease reveals neuroinflammatory acivation and biomarker candidates

Dalrymple A, Wild E, Joubert R, Sathasivam K, Björkqvist M, Petersén Å, Isaacs J, Kristiansten M, Bates G, Leavitt B, Keir G, Ward M and Tabrizi SJ.Journal of Proteome Research 6: 2833-2840 (2007)AbstractHuntington's disease (HD) causes widespread CNS changes and systemic abnormalities including endocrine and immune dysfunction. HD biomarkers are needed to power clinical trials of potential treatm

https://www.huntington-research.lu.se/proteomic-profiling-plasma-hungtintons-disease-reveals-neuroinflammatory-acivation-and-biomarker - 2025-04-29

Orexin and psychiatric symptoms in suicide attempters

Brundin L, Petersén Å, Björkqvist M and Träskman-Bendz L.Journal of Affective Disorders 100: 259-263 (2007)AbstractBACKGROUND:The orexins (hypocretins) are recently discovered hypothalamic peptides that are involved in the regulation of sleep, appetite and state of arousal. In the present study, we investigated the relationship between cerebrospinal fluid (CSF) orexin and specific psychiatric symp

https://www.huntington-research.lu.se/orexin-and-psychiatric-symptoms-suicide-attempters - 2025-04-29

Cocaine- and amphetamine-regulated transcript is increased in Huntington disease

Björkqvist M, Leavitt B, Nielsen J, Landwehrmeyer B, Ecker D, Mulder H, Brundin P and Petersén Å.Movement Disorders 22: 1952-1954 (2007)AbstractWeight loss and anxiety frequently occur in individuals with Huntington's disease (HD) but the underlying mechanisms are not well-understood. Peptides produced in the hypothalamus are involved in regulating energy homeostasis and emotion. Recent data sugge

https://www.huntington-research.lu.se/cocaine-and-amphetamine-regulated-transcript-increased-huntington-disease - 2025-04-29

Reduced orexin levels in the cerebrospinal fluid of suicidal patients with major depressive disorder

Brundin L, Björkqvist M, Petersén Å and Träskman-Bendz L.European Neuropsychopharmacology 17: 573-579 (2007)AbstractOrexins are neuropeptides selectively expressed in a small number of neurons in the lateral-posterior hypothalamus. We measured orexin-A in the cerebrospinal fluid (CSF) of 66 patients with major depressive disorder (MDD), dysthymia and adjustment disorder after a suicide attempt. Bl

https://www.huntington-research.lu.se/reduced-orexin-levels-cerebrospinal-fluid-suicidal-patients-major-depressive-disorder - 2025-04-29

Testicular degeneration in Huntington disease

Van Raamsdonk JM, Murphy Z, Selva DM, Hamidizadeh R, Pearson J, Petersen Å, Björkqvist M, Muir C, Mackenzie IR, Hammond GL, Vogl AW, Hayden MR and Leavitt BR.Neurobiology of Disease 26: 512-520 (2007)AbstractHuntington disease (HD) is an adult onset, neurodegenerative disorder that results from CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in mouse models of HD

https://www.huntington-research.lu.se/testicular-degeneration-huntington-disease - 2025-04-29

Hypothalamic-endocrine aspects in Huntington’s disease

Petersén Å and Björkqvist M.European Journal of Neuroscience 24: 961-967 (2006)AbstractHuntington's disease (HD) is a hereditary and fatal disorder caused by an expanded CAG triplet repeat in the HD gene, resulting in a mutant form of the protein huntingtin. Wild-type and mutant huntingtin are expressed in most tissues of the body but the normal function of huntingtin is not fully known. In HD, th

https://www.huntington-research.lu.se/hypothalamic-endocrine-aspects-huntingtons-disease - 2025-04-29

Quantification of Total and Mutant Huntingtin Protein Levels in Biospecimens Using a Novel alphaLISA Assay

Baldo B, Sajjad MU, Cheong RY, Bigarreau J, Vijayvargia R, McLean C, Perrier AL, Seong IS, Halliday G, Petersén Å and Kirik D.ENeuro 5 (4). pii: ENEURO.0234-18.2018 (2018)AbstractThe neurodegenerative Huntington's disease (HD) is caused by a polyglutamine (polyQ) amplification in the huntingtin protein (HTT). Currently there is no effective therapy available for HD; however, several efforts are di

https://www.huntington-research.lu.se/quantification-total-and-mutant-huntingtin-protein-levels-biospecimens-using-novel-alphalisa-assay - 2025-04-29

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

Björkqvist M, Petersén Å, Bacos K, Isaacs J, Norlén P, Gil J, Popovic N, Sundler F, Bates GP, Tabrizi SJ, Brundin P and Mulder H.Human Molecular Genetics 15: 1713-1721 (2006)AbstractHuntington's disease (HD) is characterized by a triad of motor, psychiatric and cognitive symptoms. Although many of these symptoms are likely to be related to central nervous system pathology, others may be due to cha

https://www.huntington-research.lu.se/progressive-alterations-hypothalamic-pituitary-adrenal-axis-r62-transgenic-mouse-model-huntingtons - 2025-04-29

Normal sensitivity to excitotoxicity in a transgenic Huntington’s disease rat

Winkler C, Gil JMAC, Araújo IM, Rieß O, Skripuletz I, von Hörsten S and Petersén Å.Brain Research Bulletin 69: 306-310 (2006)AbstractHuntington's disease (HD) is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the HD gene. Excitotoxic cell damage by excessive stimulation of glutamate receptors has been hypothesized to contribute to the pathogenesis of HD. Transgenic mou

https://www.huntington-research.lu.se/normal-sensitivity-excitotoxicity-transgenic-huntingtons-disease-rat - 2025-04-29

Laminin alpha1 chain improves laminin alpha2 chain deficient peripheral neuropathy

Gawlik KI, Li J-Y, Petersén Å and M Durbeej.Human Molecular Genetics 15: 2690-2700 (2006)AbstractAbsence of laminin alpha2 chain leads to a severe form of congenital muscular dystrophy (MDC1A) associated with peripheral neuropathy. Hence, future therapies should be aimed at alleviating both muscle and neurological dysfunctions. Pre-clinical studies in animal models have mainly focused on ameliorat

https://www.huntington-research.lu.se/laminin-alpha1-chain-improves-laminin-alpha2-chain-deficient-peripheral-neuropathy - 2025-04-29

Orexin loss in Huntington’s disease

Petersén Å, Gil J, Maat-Schieman MLC, Björkqvist M, Tanila H, Araujo IM, Smith R, Popovic N, Wierup N, Norlén P, Li JY, Roos RAC, Sundler F, Mulder H and Brundin P.Human Molecular Genetics 14: 39-47 (2005).AbstractHuntington's disease (HD) is a devastating neurodegenerative disorder caused by an expanded CAG repeat in the gene encoding huntingtin, a protein of unknown function. Mutant huntingtin f

https://www.huntington-research.lu.se/orexin-loss-huntingtons-disease - 2025-04-29

Evidence for dopaminergic re-innervation by embryonic allografts in an optimized rat model of the Parkinsonian variant of multiple system atrophy

Puschban Z, Stefanova N, Petersén Å, Winkler C, Brundin P, Poewe IW and Wenning GP.Brain Research Bulletin 68: 54-58 (2005)AbstractEmbryonic transplantation has been considered as an alternative treatment strategy for drug resistant parkinsonian symptoms in multiple system atrophy. So far our group has created a number of animal models of striatonigral degeneration, the core pathology underlying p

https://www.huntington-research.lu.se/evidence-dopaminergic-re-innervation-embryonic-allografts-optimized-rat-model-parkinsonian-variant - 2025-04-29

Reduced hippocampal neurogenesis in R6/2 transgenic Huntington's disease mice

Gil JM, Mohapel P, Araujo IM, Popovic N, Li JY, Brundin P and Petersen Å.Neurobiology of Disease 3: 744-751 (2005)AbstractWe investigated whether cell proliferation and neurogenesis are altered in R6/2 transgenic Huntington's disease mice. Using bromodeoxyuridine (BrdU), we found a progressive decrease in the number of proliferating cells in the dentate gyrus of R6/2 mice. This reduction was detec

https://www.huntington-research.lu.se/reduced-hippocampal-neurogenesis-r62-transgenic-huntingtons-disease-mice - 2025-04-29

Euploidy in somatic cells from R6/2 transgenic Huntington’s disease mice

Petersén Å, Stewénius Y, Björkqvist M and Gisselsson D.BMC Cell Biology 6: 34 (2005)AbstractBACKGROUND: Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the HD gene. The huntingtin protein expressed from HD has an unknown function but is suggested to interact with proteins involved in the cell division machinery. The R6/2 transgenic mouse is

https://www.huntington-research.lu.se/euploidy-somatic-cells-r62-transgenic-huntingtons-disease-mice - 2025-04-29

Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction

Smith R, Petersén Å, Bates GP, Brundin P and Li JY.Neurobiology of Disease 20: 673-84 (2005)AbstractHuntington's disease (HD) is a hereditary neurodegenerative disorder characterized by progressive psychiatric, cognitive, and motor disturbances. We studied the expression of synaptic vesicle proteins in the R6/1 transgenic mouse model of HD. We observed that the levels of rabphilin 3A, a protein in

https://www.huntington-research.lu.se/depletion-rabphilin-3a-transgenic-mouse-model-r61-huntingtons-disease-possible-culprit-synaptic - 2025-04-29

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

Björkqvist M, Fex M, Renstrom E, Wierup N, Petersén Å, Gil J, Bacos K, Popovic N, Li JY, Sundler F, Brundin P and Mulder H.Human Molecular Genetics 14: 565-574 (2005)AbstractDiabetes frequently develops in Huntington's disease (HD) patients and in transgenic mouse models of HD such as the R6/2 mouse. The underlying mechanisms have not been clarified. Elucidating the pathogenesis of diabetes in HD

https://www.huntington-research.lu.se/r62-transgenic-mouse-model-huntingtons-disease-develops-diabetes-due-deficient-beta-cell-mass-and - 2025-04-29

mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease

Ast A, Buntru A, Schindler F, Hasenkopf R, Schulz A, Brusendorf L, Klockmeier K, Grelle G, McMahon B, Niederlechner H, Jansen I, Diez L, Edel J, Boeddrich A, Franklin SA, Baldo B, Schnoegl S, Kunz S, Purfürst B, Gaertner A, Kampinga HH, Morton AJ, Petersén Å, Kirstein J, Bates GP and Wanker EE.Molecular Cell 71 (5): 675-688.(2018)AbstractSelf-propagating, amyloidogenic mutant huntingtin (mHTT) agg

https://www.huntington-research.lu.se/mhtt-seeding-activity-marker-disease-progression-and-neurotoxicity-models-huntingtons-disease - 2025-04-29