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  1. Book: The cellular and molecular biology of prion disease

    Collins, Steven J. / Lawson, Victoria A.

    2011  

    Author's details ed. Steven J. Collins ; Victoria A. Lawson
    Language English
    Size 238 S. : Ill., graph. Darst.
    Publisher Research Signpost
    Publishing place Kerala
    Publishing country India
    Document type Book
    HBZ-ID HT017382148
    ISBN 978-81-308-0446-0 ; 81-308-0446-8
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2012 A 4258 order for loan Magazin

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  2. Article ; Online: Tailored behavioural tests reveal early and progressive cognitive deficits in M1000 prion disease.

    Senesi, Matteo / Lewis, Victoria / Adlard, Paul A / Finkelstein, David I / Kim, Jee Hyun / Collins, Steven J

    Neurobiology of disease

    2023  Volume 180, Page(s) 106075

    Abstract: Prion diseases are pathogenically linked to the normal cellular prion protein ( ... ...

    Abstract Prion diseases are pathogenically linked to the normal cellular prion protein (PrP
    MeSH term(s) Animals ; Mice ; Behavior Rating Scale ; Prion Diseases/metabolism ; Cognitive Dysfunction ; Disease Progression ; Cognition
    Language English
    Publishing date 2023-03-11
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1211786-9
    ISSN 1095-953X ; 0969-9961
    ISSN (online) 1095-953X
    ISSN 0969-9961
    DOI 10.1016/j.nbd.2023.106075
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4444: Show issues Location:
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  3. Article ; Online: Comprehensive clinical, radiological, pathological and biochemical analysis required to differentiate VV1 sporadic Creutzfeldt-Jakob disease from suspected variant CJD.

    Holper, Sarah / Lewis, Victoria / Wesselingh, Robb / Gaillard, Frank / Collins, Steven J / Butzkueven, Helmut

    BMJ neurology open

    2022  Volume 4, Issue 1, Page(s) e000299

    Abstract: Background: A diagnosis of variant Creutzfeldt-Jakob disease (vCJD), the zoonotic prion disease related to transmission of bovine spongiform encephalopathy, can carry enormous public health ramifications. Until recently, all vCJD clinical cases were ... ...

    Abstract Background: A diagnosis of variant Creutzfeldt-Jakob disease (vCJD), the zoonotic prion disease related to transmission of bovine spongiform encephalopathy, can carry enormous public health ramifications. Until recently, all vCJD clinical cases were confined to patients displaying methionine homozygosity (MM) at codon 129 of the prion protein gene (
    Method and results: We present a case of CJD with clinico-epidemiological and radiological characteristics creating initial concerns for vCJD. Thorough case evaluation, including data provided by genetic testing, autopsy and neuropathological histological analyses, provided a definitive diagnosis of the rare VV1 molecular subtype of sCJD.
    Conclusion: Distinguishing vCJD from sCJD is of vital public health importance and potentially more problematic with the development of non-MM vCJD cases. The patient described herein demonstrates that in addition to the clinico-epidemiological profile, combined supplementary pathological, biochemical and critical radiological analysis may be necessary for confident discrimination of sCJD, especially rare sub-types, from vCJD.
    Language English
    Publishing date 2022-04-18
    Publishing country England
    Document type Journal Article
    ISSN 2632-6140
    ISSN (online) 2632-6140
    DOI 10.1136/bmjno-2022-000299
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Simplified Murine 3D Neuronal Cultures for Investigating Neuronal Activity and Neurodegeneration.

    Collins, Steven J / Haigh, Cathryn L

    Cell biochemistry and biophysics

    2017  Volume 75, Issue 1, Page(s) 3–13

    Abstract: The ability to model brain tissue in three-dimensions offers new potential for elucidating functional cellular interactions and corruption of such functions during pathogenesis. Many protocols now exist for growing neurones in three-dimensions and these ... ...

    Abstract The ability to model brain tissue in three-dimensions offers new potential for elucidating functional cellular interactions and corruption of such functions during pathogenesis. Many protocols now exist for growing neurones in three-dimensions and these vary in complexity and cost. Herein, we describe a straight-forward method for generating three-dimensional, terminally differentiated central nervous system cultures from adult murine neural stem cells. The protocol requires no specialist equipment, is not labour intensive or expensive and produces mature cultures within 10 days that can survive beyond a month. Populations of functional glutamatergic neurones could be identified within cultures. Additionally, the three dimensional neuronal cultures can be used to investigate tissue changes during the development of neurodegenerative disease where demonstration of hallmark features, such as plaque generation, has not previously been possible using two-dimensional cultures of neuronal cells. Using a prion model of acquired neurodegenerative disease, biochemical changes indicative of prion pathology were induced within 2-3 weeks in the three dimensional cultures. Our findings show that tissue differentiated in this simplified three dimensional culture model is physiologically competent to model central nervous system cellular behaviour as well as manifest the functional failures and pathological changes associated with neurodegenerative disease.
    MeSH term(s) Animals ; Calcium Signaling ; Cell Culture Techniques ; Cell Differentiation ; Cell Survival ; Cells, Cultured ; Mice ; Microscopy, Confocal ; Models, Neurological ; Nerve Tissue Proteins/metabolism ; Neural Stem Cells/physiology ; Neurons/metabolism ; Prion Diseases/pathology ; Spheroids, Cellular/physiology ; Thiazoles/metabolism
    Chemical Substances Nerve Tissue Proteins ; Thiazoles ; thioflavin T (2390-54-7)
    Language English
    Publishing date 2017-03
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1357904-6
    ISSN 1559-0283 ; 1085-9195
    ISSN (online) 1559-0283
    ISSN 1085-9195
    DOI 10.1007/s12013-016-0768-z
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1498: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  5. Article ; Online: Validating ASHS-T1 automated entorhinal and transentorhinal cortical segmentation in Alzheimer's disease.

    Quek, Yi-En / Bourgeat, Pierrick / Fung, Yi Leng / Vogrin, Simon J / Collins, Steven J / Bowden, Stephen C

    Psychiatry research. Neuroimaging

    2023  Volume 335, Page(s) 111707

    Abstract: The current study aimed to validate entorhinal and transentorhinal cortical volumes measured by the automated segmentation tool Automatic Segmentation of Hippocampal Subfields (ASHS-T1). The study sample comprised 34 healthy controls (HCs), 37 ... ...

    Abstract The current study aimed to validate entorhinal and transentorhinal cortical volumes measured by the automated segmentation tool Automatic Segmentation of Hippocampal Subfields (ASHS-T1). The study sample comprised 34 healthy controls (HCs), 37 individuals with amnestic mild cognitive impairment (aMCI), and 29 individuals with Alzheimer's disease (AD) dementia from the Alzheimer's Disease Neuroimaging Initiative (ADNI) database. Entorhinal and transentorhinal cortical volumes were assessed using ASHS-T1, manual segmentation, as well as a widely used automated segmentation tool, FreeSurfer v6.0.1. Mean differences, intraclass correlation coefficients, and Bland-Altman plots were computed. ASHS-T1 tended to underestimate entorhinal and transentorhinal cortical volumes relative to manual segmentation and FreeSurfer. There was variable consistency and low agreement between ASHS-T1 and manual segmentation volumes. There was low-to-moderate consistency and low agreement between ASHS-T1 and FreeSurfer volumes. There was a trend toward higher consistency and agreement for the entorhinal cortex in the aMCI and AD groups compared to the HC group. Despite the differences in volume measurements, ASHS-T1 was sensitive to entorhinal and transentorhinal cortical atrophy in both early and late disease stages. Based on the current study, ASHS-T1 appears to be a promising tool for automated entorhinal and transentorhinal cortical volume measurement in individuals with likely underlying AD.
    MeSH term(s) Humans ; Alzheimer Disease/diagnostic imaging ; Alzheimer Disease/psychology ; Image Processing, Computer-Assisted/methods ; Magnetic Resonance Imaging/methods ; Hippocampus/diagnostic imaging ; Entorhinal Cortex/diagnostic imaging
    Language English
    Publishing date 2023-08-22
    Publishing country Netherlands
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, U.S. Gov't, Non-P.H.S. ; Research Support, Non-U.S. Gov't
    ZDB-ID 445361-x
    ISSN 1872-7506 ; 1872-7123 ; 0925-4927 ; 0165-1781
    ISSN (online) 1872-7506 ; 1872-7123
    ISSN 0925-4927 ; 0165-1781
    DOI 10.1016/j.pscychresns.2023.111707
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1541: Show issues Location:
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    Jg. 1995 - 2021: Lesesall (1.OG)
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  6. Article: Alzheimer's disease biomarker utilization at first referral enhances differential diagnostic precision with simultaneous exclusion of Creutzfeldt-Jakob disease.

    Wang, Zitianyu / Lewis, Victoria / Stehmann, Christiane / Varghese, Shiji / Senesi, Matteo / McGlade, Amelia / Ellett, Laura J / Doecke, James D / Eratne, Dhamidhu / Velakoulis, Dennis / Masters, Colin L / Collins, Steven J / Li, Qiao-Xin

    Alzheimer's & dementia (Amsterdam, Netherlands)

    2024  Volume 16, Issue 1, Page(s) e12548

    Abstract: Most suspected Creutzfeldt-Jakob disease (CJD) cases are eventually diagnosed with other disorders. We assessed the utility of investigating Alzheimer's disease (AD) biomarkers and neurofilament light (NfL) in patients when CJD is suspected. The study ... ...

    Abstract Most suspected Creutzfeldt-Jakob disease (CJD) cases are eventually diagnosed with other disorders. We assessed the utility of investigating Alzheimer's disease (AD) biomarkers and neurofilament light (NfL) in patients when CJD is suspected. The study cohort consisted of cerebrospinal fluid (CSF) samples referred for CJD biomarker screening wherein amyloid beta 1-42 (Aβ1-42), phosphorylated tau 181 (p-tau181), and total tau (t-tau) could be assessed via Elecsys immunoassays (
    Language English
    Publishing date 2024-02-13
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2832898-X
    ISSN 2352-8729
    ISSN 2352-8729
    DOI 10.1002/dad2.12548
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Endoproteolytic cleavage as a molecular switch regulating and diversifying prion protein function.

    Haigh, Cathryn L / Collins, Steven J

    Neural regeneration research

    2016  Volume 11, Issue 2, Page(s) 238–239

    Language English
    Publishing date 2016-04-12
    Publishing country India
    Document type Journal Article
    ZDB-ID 2388460-5
    ISSN 1876-7958 ; 1673-5374
    ISSN (online) 1876-7958
    ISSN 1673-5374
    DOI 10.4103/1673-5374.177726
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Long-term screening for primary mitochondrial DNA variants associated with Leber hereditary optic neuropathy: incidence, penetrance and clinical features.

    Marotta, Rosetta / Chin, Judy / Chiotis, Maria / Shuey, Neil / Collins, Steven J

    Mitochondrion

    2020  Volume 54, Page(s) 128–132

    Abstract: Leber hereditary optic neuropathy (LHON) is a neurodegenerative disorder characterised by bilateral, painless, subacute, central vision loss caused by pathogenic sequence variants in mitochondrial DNA (mtDNA). Over the course of 20 years, 734 people were ...

    Abstract Leber hereditary optic neuropathy (LHON) is a neurodegenerative disorder characterised by bilateral, painless, subacute, central vision loss caused by pathogenic sequence variants in mitochondrial DNA (mtDNA). Over the course of 20 years, 734 people were systematically screened by our diagnostic laboratory for suspected LHON or for being at risk of LHON, with 98 found to harbour one of the three primary pathogenic mtDNA variants. Detection incidences were: 0.95% for NC_012920.1(MT-ND1):m.3460G>A; 9.4% for (MT-ND4):m.11778G>A; and 2.9% for (MT-ND6):m.14484T>C. The median age for symptomatic males was 27.3 years and for females 29.5 years, with a male to female ratio of 4.4:1 (62 males; 14 females). Most pathogenic variant carriers were propositi with the other individuals belonging to one of 14 pedigrees with noteworthy intra-family variability of clinical severity of the disease.
    MeSH term(s) Adolescent ; Adult ; Case-Control Studies ; DNA, Mitochondrial/genetics ; Female ; Genetic Predisposition to Disease ; Humans ; Incidence ; Male ; Middle Aged ; Mutation Rate ; NADH Dehydrogenase/genetics ; Optic Atrophy, Hereditary, Leber/genetics ; Pedigree ; Penetrance ; Polymorphism, Single Nucleotide
    Chemical Substances DNA, Mitochondrial ; NADH dehydrogenase subunit 4 ; MT-ND6 protein, human (EC 1.6.99.3) ; NADH Dehydrogenase (EC 1.6.99.3) ; MT-ND1 protein, human (EC 7.1.1.2)
    Language English
    Publishing date 2020-08-28
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2056923-3
    ISSN 1872-8278 ; 1567-7249
    ISSN (online) 1872-8278
    ISSN 1567-7249
    DOI 10.1016/j.mito.2020.08.007
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Retinoic acid receptors, hematopoiesis and leukemogenesis.

    Collins, Steven J

    Current opinion in hematology

    2008  Volume 15, Issue 4, Page(s) 346–351

    Abstract: Purpose of review: All-trans retinoic acid therapy of acute promyelocytic leukemia represents the most successful example of differentiation-induction therapy in clinical oncology. However, acute promyelocytic leukemia represents only a small minority ( ... ...

    Abstract Purpose of review: All-trans retinoic acid therapy of acute promyelocytic leukemia represents the most successful example of differentiation-induction therapy in clinical oncology. However, acute promyelocytic leukemia represents only a small minority (10-15%) of the myeloid leukemias. Recent studies provide significant insight into why some myeloid leukemias respond dramatically to all-trans retinoic acid mediated differentiation therapy, whereas others do not.
    Recent findings: Utilizing in-vitro experimental models of all-trans retinoic acid triggered myeloid leukemia differentiation, specific genes that are important regulators of granulocytic differentiation have been identified including transcription factors, apoptosis regulators, protein synthesis inhibitors and protein degradation factors. Moreover, recent studies have identified repressive chromatin marks generated by the aberrant, acute promyelocytic leukemia specific promyelocytic locus gene-retinoic acid receptor alpha (PML-RARalpha) fusion protein as well as the specific enzymes that mediate these chromatin changes.
    Summary: The molecular basis for PML-RARalpha- mediated leukemogenesis is complex involving both the repression of numerous potential target genes and critical 'off promoter' functional activity of this fusion protein. The acute promyelocytic leukemia specific repressive chromatin marks related to PML-RARalpha activity may be present in other myeloid leukemias as well. This suggests alternative approaches for treating myeloid leukemia involving therapeutic agents that inhibit heterochromatin formation and enhance transcriptional activity. All-trans retinoic acid or related compounds may also play a significant role in enhancing hematopoietic stem cell self-renewal as well as the production and differentiation of regulatory T cells.
    MeSH term(s) Animals ; Cell Differentiation/drug effects ; Hematopoiesis ; Humans ; Leukemia, Myeloid/drug therapy ; Leukemia, Myeloid/etiology ; Leukemia, Promyelocytic, Acute/drug therapy ; Leukemia, Promyelocytic, Acute/etiology ; Receptors, Retinoic Acid ; Tretinoin/pharmacology ; Tretinoin/therapeutic use
    Chemical Substances Receptors, Retinoic Acid ; Tretinoin (5688UTC01R)
    Language English
    Publishing date 2008-07
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1153887-9
    ISSN 1531-7048 ; 1065-6251
    ISSN (online) 1531-7048
    ISSN 1065-6251
    DOI 10.1097/MOH.0b013e3283007edf
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 3703: Show issues Location:
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  10. Article: Diagnostic performance of CSF biomarkers in a well-characterized Australian cohort of sporadic Creutzfeldt-Jakob disease.

    Senesi, Matteo / Lewis, Victoria / Varghese, Shiji / Stehmann, Christiane / McGlade, Amelia / Doecke, James D / Ellett, Laura / Sarros, Shannon / Fowler, Christopher J / Masters, Colin L / Li, Qiao-Xin / Collins, Steven J

    Frontiers in neurology

    2023  Volume 14, Page(s) 1072952

    Abstract: The most frequently utilized biomarkers to support a pre-mortem clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) include concentrations of the 14-3-3 and total tau (T-tau) proteins, as well as the application of protein amplification ... ...

    Abstract The most frequently utilized biomarkers to support a pre-mortem clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) include concentrations of the 14-3-3 and total tau (T-tau) proteins, as well as the application of protein amplification techniques, such as the real time quaking-induced conversion (RT-QuIC) assay, in cerebrospinal fluid (CSF). Utilizing CSF from a cohort of neuropathologically confirmed (definite) sCJD (
    Language English
    Publishing date 2023-02-08
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2564214-5
    ISSN 1664-2295
    ISSN 1664-2295
    DOI 10.3389/fneur.2023.1072952
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