Skip to main content

Research Repository

Advanced Search

Outputs (64)

Proteomic characterization of human LMNA-related congenital muscular dystrophy muscle cells (2024)
Journal Article
Storey, E., Holt, I., Brown, S., Synowsky, S., Shirran, S., & Fuller, H. (2024). Proteomic characterization of human LMNA-related congenital muscular dystrophy muscle cells. Neuromuscular Disorders, 38, 26-41. https://doi.org/10.1016/j.nmd.2024.03.006

LMNA-related congenital muscular dystrophy (L-CMD) is caused by mutations in the LMNA gene, encoding lamin A/C. To further understand the molecular mechanisms of L-CMD, proteomic profiling using DIA mass spectrometry was conducted on immortalized myo... Read More about Proteomic characterization of human LMNA-related congenital muscular dystrophy muscle cells.

Increased NLRP1 mRNA and Protein Expression Suggests Inflammasome Activation in the Dorsolateral Prefrontal and Medial Orbitofrontal Cortex in Schizophrenia (2024)
Journal Article
Španić Popovački, E., Vogrinc, D., Fuller, H., Langer Horvat, L., Mayer, D., Kopić, J., … Šimić, G. (2024). Increased NLRP1 mRNA and Protein Expression Suggests Inflammasome Activation in the Dorsolateral Prefrontal and Medial Orbitofrontal Cortex in Schizophrenia. Biomolecules, 14(3), Article 302. https://doi.org/10.3390/biom14030302

Schizophrenia is a complex mental condition, with key symptoms marked for diagnosis including delusions, hallucinations, disorganized thinking, reduced emotional expression, and social dysfunction. In the context of major developmental hypotheses of... Read More about Increased NLRP1 mRNA and Protein Expression Suggests Inflammasome Activation in the Dorsolateral Prefrontal and Medial Orbitofrontal Cortex in Schizophrenia.

AAV9-mediated SMN gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in Smn2B/- spinal muscular atrophy mice Human Molecular Genetics (2023)
Journal Article
Brown, S., Šoltić, D., Synowsky, S. A., Shirran, S. L., Chilcott, E., Shorrock, H. K., …Fuller, H. (2023). AAV9-mediated SMN gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in Smn2B/- spinal muscular atrophy mice Human Molecular Genetics. Human Molecular Genetics, Article ddad121. https://doi.org/10.1093/hmg/ddad121

Structural, functional and molecular cardiac defects have been reported in spinal muscular atrophy (SMA) patients and mouse models. Previous quantitative proteomics analyses demonstrated widespread molecular defects in the severe Taiwanese SMA mouse... Read More about AAV9-mediated SMN gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in Smn2B/- spinal muscular atrophy mice Human Molecular Genetics.

Enhanced expression of the human Survival motor neuron 1 gene from a sequence-optimised cDNA transgene in vitro and in vivo (2023)
Journal Article
Nafchi, N., Chilcott, E., Owen, S., Fuller, H., Bowerman, M., & Yáñez-Muñoz, R. (in press). Enhanced expression of the human Survival motor neuron 1 gene from a sequence-optimised cDNA transgene in vitro and in vivo. Gene Therapy, https://doi.org/10.1038/s41434-023-00406-0

Spinal muscular atrophy (SMA) is a neuromuscular disease particularly characterised by degeneration of ventral motor neurons. Survival motor neuron (SMN) 1 gene mutations cause SMA, and gene addition strategies to replace the faulty SMN1 copy are a t... Read More about Enhanced expression of the human Survival motor neuron 1 gene from a sequence-optimised cDNA transgene in vitro and in vivo.

Enhanced expression of the human Survival motor neuron 1 gene from a codon-optimised cDNA transgene in vitro and in vivo (2023)
Journal Article
Nafchi, N., Chilcott, E., Brown, S., Fuller, H., Bowerman, M., & Yáñez-Muñoz, R. (2023). Enhanced expression of the human Survival motor neuron 1 gene from a codon-optimised cDNA transgene in vitro and in vivo. Gene Therapy, https://doi.org/10.1038/s41434-023-00406-0

Spinal muscular atrophy (SMA) is a neuromuscular disease particularly characterised by degeneration of ventral motor neurons. Survival motor neuron (SMN) 1 gene mutations cause SMA, and gene addition strategies to replace the faulty SMN1 copy are a t... Read More about Enhanced expression of the human Survival motor neuron 1 gene from a codon-optimised cDNA transgene in vitro and in vivo.

Genotype-phenotype correlations in human diseases caused by mutations of LINC complex-associated genes: a systematic review and meta-summary (2022)
Journal Article
Fuller, H., & Storey, E. (2022). Genotype-phenotype correlations in human diseases caused by mutations of LINC complex-associated genes: a systematic review and meta-summary. Cells, https://doi.org/10.3390/cells11244065

Mutations in genes encoding proteins associated with the linker of nucleoskel-eton and cytoskeleton (LINC) complex within the nuclear envelope cause dif-ferent diseases with varying phenotypes including skeletal muscle, cardiac, metabolic, or nervous... Read More about Genotype-phenotype correlations in human diseases caused by mutations of LINC complex-associated genes: a systematic review and meta-summary.

A Meta-summary and Bioinformatic Analysis Identified Interleukin 6 as a Master Regulator of COVID-19 Severity Biomarkers (2022)
Journal Article
Ghanem, M., Owen, S., Mohamed, A., & Fuller, H. (2022). A Meta-summary and Bioinformatic Analysis Identified Interleukin 6 as a Master Regulator of COVID-19 Severity Biomarkers. Cytokine, 159, Article 156011. https://doi.org/10.1016/j.cyto.2022.156011

With the rising demand for improved COVID-19 disease monitoring and prognostic markers, studies have aimed to identify biomarkers using a range of screening methods. However, the selection of biomarkers for validation from large datasets may result i... Read More about A Meta-summary and Bioinformatic Analysis Identified Interleukin 6 as a Master Regulator of COVID-19 Severity Biomarkers.

The proteome signatures of fibroblasts from patients with severe, intermediate and mild spinal muscular atrophy show limited overlap (2022)
Journal Article
Owen, S., Kline, R., Synowsky, S., Shirran, S., Sillence, K., Claus, P., …Holt, I. (2022). The proteome signatures of fibroblasts from patients with severe, intermediate and mild spinal muscular atrophy show limited overlap. Cells, https://doi.org/10.3390/cells11172624

Most research to characterise the molecular consequences of spinal muscular atrophy (SMA) have focused on SMA I. Here, proteomic profiling of skin fibroblasts from severe (SMA I), intermediate (SMA II), and mild (SMA III) patients, alongside age-matc... Read More about The proteome signatures of fibroblasts from patients with severe, intermediate and mild spinal muscular atrophy show limited overlap.

An Anatomy of the Blood Eagle: The Practicalities of Viking Torture (2022)
Journal Article
Murphy, L. J., Fuller, H. R., Willan, P. L., & Gates, M. (2022). An Anatomy of the Blood Eagle: The Practicalities of Viking Torture. Speculum, 1-39. https://doi.org/10.1086/717332

The infamous blood eagle ritual has long been controversial: did Viking-Age Nordic people really torture one another to death by severing their ribs from their spine and removing their lungs, or is it all a misunderstanding of some complicated poetry... Read More about An Anatomy of the Blood Eagle: The Practicalities of Viking Torture.

SMA - TREATMENT (2021)
Journal Article
Owen, S., Šoltić, D., Synowsky, S., Crompton, E., Yáñez-Muñoz, R., Schneider, B., …Fuller, H. (2021). SMA - TREATMENT. Neuromuscular Disorders, 31, S131-S132. https://doi.org/10.1016/j.nmd.2021.07.295

Proteins associated with the sarcomere and costamere in hearts are dysregulated in two mouse models of spinal muscular atrophy

Investigation of the blood proteome in response to spinal cord injury in rodent models (2021)
Journal Article
Hulme, C. H., Fuller, H. R., Riddell, J., Shirran, S. L., Botting, C. H., Osman, A., & Wright, K. T. (2022). Investigation of the blood proteome in response to spinal cord injury in rodent models. Spinal Cord, 60(4), 320-325. https://doi.org/10.1038/s41393-021-00692-8

STUDY DESIGN: Explanatory and Mechanistic Study OBJECTIVES: A better understanding of the ‘whole-body’ response following spinal cord injury (SCI) is needed to guide future research aimed at developing novel therapeutic interventions and identifying... Read More about Investigation of the blood proteome in response to spinal cord injury in rodent models.

An interaction of heart disease-associated proteins POPDC1/2 with XIRP1 in transverse tubules and intercalated discs. (2020)
Journal Article
Holt, I., Fuller, H. R., Schindler, R. F., Shirran, S. L., Brand, T., & Morris, G. E. (2020). An interaction of heart disease-associated proteins POPDC1/2 with XIRP1 in transverse tubules and intercalated discs. https://doi.org/10.1186/s12860-020-00329-3

BACKGROUND: Popeye domain-containing proteins 1 and 2 (POPDC1 and POPDC2) are transmembrane proteins involved in cyclic AMP-mediated signalling processes and are required for normal cardiac pacemaking and conduction. In order to identify novel protei... Read More about An interaction of heart disease-associated proteins POPDC1/2 with XIRP1 in transverse tubules and intercalated discs..

Muscle cell differentiation and development pathway defects in Emery-Dreifuss muscular dystrophy (2020)
Journal Article
Storey, E., Holt, I., Morris, G., & Fuller, H. (2020). Muscle cell differentiation and development pathway defects in Emery-Dreifuss muscular dystrophy. Neuromuscular Disorders, 443-456. https://doi.org/10.1016/j.nmd.2020.04.002

Emery-Dreifuss muscular dystrophy (EDMD) is a rare genetic disorder characterised by the early development of muscle contractures, progressive muscle weakness, and heart abnormalities. The latter may result in serious complications, or in severe case... Read More about Muscle cell differentiation and development pathway defects in Emery-Dreifuss muscular dystrophy.

Quantitative proteomic profiling of the rat substantia nigra places glial fibrillary acidic protein at the hub of proteins dysregulated during aging: Implications for idiopathic Parkinson’s disease (2020)
Journal Article
Gómez-Gálvez, Y., Fuller, H., Synowsky, S., Shirran, L., & Gates, M. (2020). Quantitative proteomic profiling of the rat substantia nigra places glial fibrillary acidic protein at the hub of proteins dysregulated during aging: Implications for idiopathic Parkinson’s disease. Journal of Neuroscience Research, 1417-1432. https://doi.org/10.1002/jnr.24622

There is a strong correlation between aging and onset of idiopathic Parkinson’s disease, but little is known about whether cellular changes occur during normal aging that may explain this association. Here, proteomic and bioinformatic analysis was co... Read More about Quantitative proteomic profiling of the rat substantia nigra places glial fibrillary acidic protein at the hub of proteins dysregulated during aging: Implications for idiopathic Parkinson’s disease.

Molecular crosstalk between non-SMN-related and SMN-related spinal muscular atrophy (2020)
Journal Article
Šoltić, D., & Fuller, H. (2020). Molecular crosstalk between non-SMN-related and SMN-related spinal muscular atrophy

Most cases of spinal muscular atrophy are caused by functional loss of the survival of motor neuron 1 (SMN1) gene, while less than 5% of cases are attributed to genes other than SMN. Mutations in LMNA, the lamin A/C encoding gene, cause an adult form... Read More about Molecular crosstalk between non-SMN-related and SMN-related spinal muscular atrophy.

Lamin A/C dysregulation contributes to cardiac pathology in a mouse model of severe spinal muscular atrophy (2019)
Journal Article
Šoltić, D., Shorrock, H. K., Allardyce, H., Wilson, E. L., Holt, I., Synowsky, S. A., …Fuller, H. (2019). Lamin A/C dysregulation contributes to cardiac pathology in a mouse model of severe spinal muscular atrophy. Human Molecular Genetics, 3515-3527. https://doi.org/10.1093/hmg/ddz195

Cardiac pathology is emerging as a prominent systemic feature of spinal muscular atrophy (SMA), but little is known about the underlying molecular pathways. Using quantitative proteomics analysis, we demonstrate widespread molecular defects in heart... Read More about Lamin A/C dysregulation contributes to cardiac pathology in a mouse model of severe spinal muscular atrophy.

Nesprin-1-alpha2 associates with kinesin at myotube outer nuclear membranes, but is restricted to neuromuscular junction nuclei in adult muscle (2019)
Journal Article
Holt, I., Fuller, H., Lam, L., Sewry, C., Shirran, S., Zhang, Q., …Morris, G. (2019). Nesprin-1-alpha2 associates with kinesin at myotube outer nuclear membranes, but is restricted to neuromuscular junction nuclei in adult muscle. Scientific reports, https://doi.org/10.1038/s41598-019-50728-6

Nesprins, nuclear envelope spectrin-repeat proteins encoded by the SYNE1 and SYNE2 genes, are involved in localization of nuclei. The short isoform, nesprin-1-alpha2, is required for relocation of the microtubule organizer function from centromeres t... Read More about Nesprin-1-alpha2 associates with kinesin at myotube outer nuclear membranes, but is restricted to neuromuscular junction nuclei in adult muscle.

Breast reconstruction affects coping mechanisms in breast cancer survivors. (2019)
Journal Article
Lake, B., Fuller, H., Rastall, S., & Usman, T. (2019). Breast reconstruction affects coping mechanisms in breast cancer survivors. Indian Journal of Surgery, 43-50. https://doi.org/10.1007/s12262-017-1712-1

Coping strategies used by women with breast cancer are vital for adjustment to their disease. Whilst it is clear that factors such as age at diagnosis, social support and ethnicity can influence coping mechanisms, there is currently no information ab... Read More about Breast reconstruction affects coping mechanisms in breast cancer survivors..

Multi-Study Proteomic and Bioinformatic Identification of Molecular Overlap between Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA) (2018)
Journal Article
Šoltić, D., Bowerman, M., Stock, J., Shorrock, H. K., Gillingwater, T. H., & Fuller, H. R. (2018). Multi-Study Proteomic and Bioinformatic Identification of Molecular Overlap between Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA). Brain Sciences, https://doi.org/10.3390/brainsci8120212

Unravelling the complex molecular pathways responsible for motor neuron degeneration in amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) remains a persistent challenge. Interest is growing in the potential molecular similarities... Read More about Multi-Study Proteomic and Bioinformatic Identification of Molecular Overlap between Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA).

A systematic review and meta-analysis of the effectiveness of surgical decompression in treating patients with malignant middle cerebral artery infarction (2018)
Journal Article
Gul, W., Fuller, H., Sen, J., & Wright, H. (2018). A systematic review and meta-analysis of the effectiveness of surgical decompression in treating patients with malignant middle cerebral artery infarction. World Neurosurgery, 120, e902-e920. https://doi.org/10.1016/j.wneu.2018.08.189

Background Malignant infarction of the middle-cerebral-artery (MCI) is life threatening. It is associated with a mortality as high as 80% and survival often at the expense of serious disability. Limited success of medical therapies has resulted in d... Read More about A systematic review and meta-analysis of the effectiveness of surgical decompression in treating patients with malignant middle cerebral artery infarction.

Two independent proteomic approaches provide a comprehensive analysis of the synovial fluid proteome response to Autologous Chondrocyte Implantation (2018)
Journal Article
Hulme, C., Wilson, E., Fuller, H., Roberts, S., Richardson, J., Gallacher, P., …Wright, K. (2018). Two independent proteomic approaches provide a comprehensive analysis of the synovial fluid proteome response to Autologous Chondrocyte Implantation. Arthritis Research and Therapy, 20, Article 87. https://doi.org/10.1186/s13075-018-1573-4

Background Autologous chondrocyte implantation (ACI) has a failure rate of approximately 20%, but it is yet to be fully understood why. Biomarkers are needed that can pre-operatively predict in which patients it is likely to fail, so that alternativ... Read More about Two independent proteomic approaches provide a comprehensive analysis of the synovial fluid proteome response to Autologous Chondrocyte Implantation.

Proteomic analysis of age-related changes in ovine cerebrospinal fluid (2018)
Journal Article
Chen, C., Preston, J., Zhou, S., Fuller, H., Morgan, D., & Chen, R. (2018). Proteomic analysis of age-related changes in ovine cerebrospinal fluid. Experimental Gerontology, 108, 181-188. https://doi.org/10.1016/j.exger.2018.04.012

Cerebrospinal fluid (CSF) circulates through the brain and has a unique composition reflecting the biological processes of the brain. Identifying ageing CSF biomarkers can aid in understanding the ageing process and interpreting CSF protein changes i... Read More about Proteomic analysis of age-related changes in ovine cerebrospinal fluid.

Acute phase response signalling is altered following cartilage harvest in non-responders to Autologous Chondrocyte Implantation (2018)
Journal Article
Hulme, C., Wilson, E., Fuller, H., Roberts, S., Richardson, J., Gallacher, P., …Wright, K. (2018). Acute phase response signalling is altered following cartilage harvest in non-responders to Autologous Chondrocyte Implantation. Osteoarthritis and Cartilage, 26(S1), S167. https://doi.org/10.1016/j.joca.2018.02.363

Background: Autologous chondrocyte implantation (ACI) has a failure rate of approximately 20%, but it is yet to be fully understood why. Biomarkers are needed that can pre-operatively predict in which patients it is likely to fail, so that alternat... Read More about Acute phase response signalling is altered following cartilage harvest in non-responders to Autologous Chondrocyte Implantation.

Two cases of spinal muscular atrophy type II with eosinophilic oesophagitis (2017)
Journal Article
Fuller, H. R., Shorrock, H. K., Gillingwater, T. H., Pigott, A., Smith, V., Kulshrestha, R., …Willis, T. A. (2017). Two cases of spinal muscular atrophy type II with eosinophilic oesophagitis. Journal of Neuromuscular Diseases, 357-362

Although primarily characterised by loss of motor neurons from the anterior horn of spinal cord and muscle atrophy, spinal muscular atrophy (SMA) is now recognised as a multi-systemic disorder. Here, we report two SMA Type II patients with eosinophil... Read More about Two cases of spinal muscular atrophy type II with eosinophilic oesophagitis.

Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo (2017)
Journal Article
Llavero Hurtado, M., Fuller, H., Wong, A., Eaton, S., Gillingwater, T., Pennetta, G., …Wishart, T. (2017). Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo. Scientific reports, https://doi.org/10.1038/s41598-017-12603-0

Synapses are an early pathological target in many neurodegenerative diseases ranging from well-known adult onset conditions such as Alzheimer and Parkinson disease to neurodegenerative conditions of childhood such as spinal muscular atrophy (SMA) and... Read More about Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo.

We may not be buying what the label says on the tin: a cautionary tale from a study of the influence of proteoglycans on nerve growth (2017)
Journal Article
Owen, S., Fuller, H., Jones, P., Caterson, B., Shirran, S., Botting, C., & Roberts, S. (2017). We may not be buying what the label says on the tin: a cautionary tale from a study of the influence of proteoglycans on nerve growth. International Journal of Experimental Pathology, 98(3), A4

Introduction Commercially sourced preparations of reagents are an essential tool for all our laboratory studies and are assumed to have a certain degree of purity. During a study to determine how different proteoglycan substrates might control ner... Read More about We may not be buying what the label says on the tin: a cautionary tale from a study of the influence of proteoglycans on nerve growth.

The Developing Landscape of Diagnostic and Prognostic Biomarkers for Spinal Cord Injury in Cerebrospinal Fluid and Blood (2016)
Journal Article
Hulme, C., Brown, S., Fuller, H., Riddell, J., Osman, A., Chowdhury, J., …Wright, K. (2017). The Developing Landscape of Diagnostic and Prognostic Biomarkers for Spinal Cord Injury in Cerebrospinal Fluid and Blood. Spinal Cord, 55, 114-125. https://doi.org/10.1038/sc.2016.174

Objectives: The identification of prognostic biomarkers of spinal cord injury (SCI) will help to assign SCI patients to the correct treatment and rehabilitation regimes. Further, the detection of biomarkers that predict permanent neurological outcom... Read More about The Developing Landscape of Diagnostic and Prognostic Biomarkers for Spinal Cord Injury in Cerebrospinal Fluid and Blood.

Contaminants in Commercial Preparations of ‘Purified’ Small Leucine-Rich Proteoglycans May Distort Mechanistic Studies (2016)
Journal Article
Owen, S., Jones, P., Caterson, B., Shirran, S., Fuller, H., Botting, C., & Roberts, S. (2016). Contaminants in Commercial Preparations of ‘Purified’ Small Leucine-Rich Proteoglycans May Distort Mechanistic Studies. Bioscience Reports, https://doi.org/10.1042/BSR20160465

This paper reports the perplexing results that came about because of seriously impure commercially available reagents. Commercial reagents and chemicals are routinely ordered by scientists and are expected to have been rigorously assessed for their p... Read More about Contaminants in Commercial Preparations of ‘Purified’ Small Leucine-Rich Proteoglycans May Distort Mechanistic Studies.

Gene expression profiling of the dorsolateral and medial orbitofrontal cortex in schizophrenia (2016)
Journal Article
Mladinov, M., Sedmak, G., Fuller, H., Babić Leko, M., Mayer, D., Kirincich, J., …Šimić, G. (2016). Gene expression profiling of the dorsolateral and medial orbitofrontal cortex in schizophrenia. Translational Neuroscience, https://doi.org/10.1515/tnsci-2016-0021

Schizophrenia is a complex polygenic disorder of unknown etiology. Over 3,000 candidate genes associated with schizophrenia have been reported, most of which being mentioned only once. Alterations in cognitive processing - working memory, metacogniti... Read More about Gene expression profiling of the dorsolateral and medial orbitofrontal cortex in schizophrenia.

Cross-Reactivity between Schistosoma mansoni Antigens and the Latex Allergen Hev b 7: Putative Implication of Cross-Reactive Carbohydrate Determinants (CCDs) (2016)
Journal Article
Liddell, S., Fuller, H., Stanley, R., Schramm, G., Igetei, J., Doenhoff, M., & El-Faham, M. (2016). Cross-Reactivity between Schistosoma mansoni Antigens and the Latex Allergen Hev b 7: Putative Implication of Cross-Reactive Carbohydrate Determinants (CCDs). PloS one, https://doi.org/10.1371/journal.pone.0159542

IgG antibodies produced by rabbits immunized against S. mansoni antigens cross-reacted with aqueous soluble constituents of a variety of allergens. The antibody cross-reactivity was largely sensitive to degradation by treatment of the target antigens... Read More about Cross-Reactivity between Schistosoma mansoni Antigens and the Latex Allergen Hev b 7: Putative Implication of Cross-Reactive Carbohydrate Determinants (CCDs).

Understanding the molecular consequences of inherited muscular dystrophies: advancements through proteomic experimentation (2016)
Journal Article
Fuller, H., Graham, L., Llavero Hurtado, M., & Wishart, T. (2016). Understanding the molecular consequences of inherited muscular dystrophies: advancements through proteomic experimentation. Expert Review of Proteomics, 659-671. https://doi.org/10.1080/14789450.2016.1202768

Introduction: Proteomic techniques offer insights into the molecular perturbations occurring in muscular-dystrophies (MD). Revisiting published datasets can highlight conserved downstream molecular alterations, which may be worth re-assessing to dete... Read More about Understanding the molecular consequences of inherited muscular dystrophies: advancements through proteomic experimentation.

Monoclonal antibody Py recognizes neurofilament heavy chain and is a selective marker for large diameter neurons in the brain Brain Structure and Function (2016)
Journal Article
Fuller, H. R., Marani, L., Holt, I., Woodhams, P. L., Webb, M. M., & Gates, M. A. (2016). Monoclonal antibody Py recognizes neurofilament heavy chain and is a selective marker for large diameter neurons in the brain Brain Structure and Function. Brain Structure and Function, 807-879. https://doi.org/10.1007/s00429-016-1252-7

Almost 30 years ago, the monoclonal antibody Py was developed to detect pyramidal neurons in the CA3 region of the rat hippocampus. The utility of this antibody quickly expanded when several groups discovered that it could be used to identify very sp... Read More about Monoclonal antibody Py recognizes neurofilament heavy chain and is a selective marker for large diameter neurons in the brain Brain Structure and Function.

Commonality amid diversity: multi-study proteomic identification of conserved disease mechanisms in spinal muscular atrophy (2016)
Journal Article
Fuller, H., Wishart, T., & Gillingwater, T. (2016). Commonality amid diversity: multi-study proteomic identification of conserved disease mechanisms in spinal muscular atrophy. Neuromuscular Disorders, 560-569. https://doi.org/10.1016/j.nmd.2016.06.004

The neuromuscular disease spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality, resulting from low levels of full-length survival motor neuron (SMN) protein. Despite having a good understanding of the underlying genetics of SM... Read More about Commonality amid diversity: multi-study proteomic identification of conserved disease mechanisms in spinal muscular atrophy.

Anti-epileptic drugs and bone loss: phenytoin reduces pro-collagen I and alters the electrophoretic mobility of osteonectin in cultured bone cells (2016)
Journal Article
Wilson, E., Garton, M., & Fuller, H. (2016). Anti-epileptic drugs and bone loss: phenytoin reduces pro-collagen I and alters the electrophoretic mobility of osteonectin in cultured bone cells. Epilepsy Research, 97-101. https://doi.org/10.1016/j.eplepsyres.2016.03.002

Phenytoin is an antiepileptic drug used in the management of partial and tonic-clonic seizures. In previous studies we have shown that valproate, another antiepileptic drug, reduced the amount of two key bone proteins, pro-collagen I and osteonectin... Read More about Anti-epileptic drugs and bone loss: phenytoin reduces pro-collagen I and alters the electrophoretic mobility of osteonectin in cultured bone cells.

Abstract P4-10-14: Breast reconstruction changes coping mechanisms in breast cancer survivorship (2016)
Journal Article
Lake, B., Fuller, H., Rastall, S., & Usman, T. (2016). Abstract P4-10-14: Breast reconstruction changes coping mechanisms in breast cancer survivorship. Cancer Research, 76(4_Supplement), P4-10-14-P4-10-14. https://doi.org/10.1158/1538-7445.sabcs15-p4-10-14

Introduction Cancer survivorship is the process of living through and beyond cancer; a key part is how a patient copes with their diagnosis. Breast cancer is the most common malignancy of women worldwide and is known to be a severe stressor. Resea... Read More about Abstract P4-10-14: Breast reconstruction changes coping mechanisms in breast cancer survivorship.

Spinal muscular atrophy patient iPSC-derived motor neurons have reduced expression of proteins important in neuronal development (2016)
Journal Article
Fuller, H., Mandefro, B., Shirran, S., Gross, A., Kaus, A., Botting, C., …Sareen, S. (2016). Spinal muscular atrophy patient iPSC-derived motor neurons have reduced expression of proteins important in neuronal development. Frontiers in Cellular Neuroscience, https://doi.org/10.3389/fncel.2015.00506

Spinal muscular atrophy (SMA) is an inherited neuromuscular disease primarily characterized by degeneration of spinal motor neurons, and caused by reduced levels of the SMN protein. Previous studies to understand the proteomic consequences of reduced... Read More about Spinal muscular atrophy patient iPSC-derived motor neurons have reduced expression of proteins important in neuronal development.

Human mesenchymal stem cells stimulate EaHy926 endothelial cell migration: combined proteomic and in vitro analysis of the influence of donor-donor variability (2015)
Journal Article
Walter, M., Kohli, N., Major, T., Fuller, H., Wright, K., Kuiper, J., & Johnson, W. (2015). Human mesenchymal stem cells stimulate EaHy926 endothelial cell migration: combined proteomic and in vitro analysis of the influence of donor-donor variability. Journal of Stem Cells and Regenerative Medicine, 11(1), 18-24. https://doi.org/10.46582/jsrm.1101004

Mesenchymal stem cells (MSCs) stimulate angiogenesis within a wound environment and this effect is mediated through paracrine interactions with the endothelial cells present. Here we report that human MSC-conditioned medium (n=3 donors) significantly... Read More about Human mesenchymal stem cells stimulate EaHy926 endothelial cell migration: combined proteomic and in vitro analysis of the influence of donor-donor variability.

The influence of storage parameters on measurement of survival motor neuron (SMN) protein levels: Implications for pre-clinical studies and clinical trials for spinal muscular atrophy (2014)
Journal Article
Hunter, G., Roche, S. L., Somers, E., Fuller, H. R., & Gillingwater, T. H. (2014). The influence of storage parameters on measurement of survival motor neuron (SMN) protein levels: Implications for pre-clinical studies and clinical trials for spinal muscular atrophy. Neuromuscular Disorders, 24(11), 973-977. https://doi.org/10.1016/j.nmd.2014.05.013

The rat striatum responds to nigro-striatal degeneration via the increased expression of proteins associated with growth and regeneration of neuronal circuitry (2014)
Journal Article
Fuller, H. R., Hurtado, M. L., Wishart, T. M., & Gates, M. A. (2014). The rat striatum responds to nigro-striatal degeneration via the increased expression of proteins associated with growth and regeneration of neuronal circuitry. Proteome Science, 1-20. https://doi.org/10.1186/1477-5956-12-20

BACKGROUND: Idiopathic Parkinson's disease is marked by degeneration of dopamine neurons projecting from the substantia nigra to the striatum. Although proteins expressed by the target striatum can positively affect the viability and growth of dopami... Read More about The rat striatum responds to nigro-striatal degeneration via the increased expression of proteins associated with growth and regeneration of neuronal circuitry.

Dysregulation of ubiquitin homeostasis and ß-catenin signaling promote spinal muscular atrophy (2014)
Journal Article
Wishart, T., Becker, C., Wirth, B., Gillingwater, T., Parson, S., Skehel, P., …Morris, G. (2014). Dysregulation of ubiquitin homeostasis and ß-catenin signaling promote spinal muscular atrophy. Journal of Clinical Investigation, 1821 -1834. https://doi.org/10.1172/JCI71318

The autosomal recessive neurodegenerative disease spinal muscular atrophy (SMA) results from low levels of survival motor neuron (SMN) protein; however, it is unclear how reduced SMN promotes SMA development. Here, we determined that ubiquitin-depend... Read More about Dysregulation of ubiquitin homeostasis and ß-catenin signaling promote spinal muscular atrophy.

Naturally occurring plant polyphenols as potential therapies for inherited neuromuscular diseases (2013)
Journal Article
Fuller, H. R., Humphrey, E. L., & Morris, G. E. (2013). Naturally occurring plant polyphenols as potential therapies for inherited neuromuscular diseases. Future Medicinal Chemistry, 5(17), 2091-2101. https://doi.org/10.4155/fmc.13.165

There are several lines of laboratory-based evidence emerging to suggest that purified polyphenol compounds such as resveratrol, found naturally in red grapes, epigallocatechin galate from green tea and curcumin from turmeric, might be useful for the... Read More about Naturally occurring plant polyphenols as potential therapies for inherited neuromuscular diseases.

The gemin2-binding site on SMN protein: Accessibility to antibody (2013)
Journal Article
Lam, L. T., Fuller, H. R., & Morris, G. E. (2013). The gemin2-binding site on SMN protein: Accessibility to antibody. Biochemical and Biophysical Research Communications, 438(4), 624-627. https://doi.org/10.1016/j.bbrc.2013.08.005

Reduced levels of SMN (survival-of-motor-neurons) protein are the cause of spinal muscular atrophy, an inherited disorder characterised by loss of motor neurons in early childhood. SMN associates with more than eight other proteins to form an RNA-bin... Read More about The gemin2-binding site on SMN protein: Accessibility to antibody.

Valproate reduces collagen and osteonectin in cultured bone cells (2013)
Journal Article
Humphrey, E. L., Morris, G. E., & Fuller, H. R. (2013). Valproate reduces collagen and osteonectin in cultured bone cells. Epilepsy Research, 106(3), 446-450. https://doi.org/10.1016/j.eplepsyres.2013.06.011

Valproate is a histone deacetylase (HDAC) inhibitor that was introduced more than 40 years ago and is commonly used to treat epilepsy and mood disorders. Its long-term side effects can include bone loss, although the exact mechanism for this is curre... Read More about Valproate reduces collagen and osteonectin in cultured bone cells.

ApoE isoform-specific regulation of regeneration in the peripheral nervous system (2011)
Journal Article
Comley, L. H., Fuller, H. R., Wishart, T. M., Mutsaers, C. A., Thomson, D., Wright, A. K., …Gillingwater, T. H. (2011). ApoE isoform-specific regulation of regeneration in the peripheral nervous system. Human molecular genetics, 20(12), 2406-2421. https://doi.org/10.1093/hmg/ddr147

Apolipoprotein E (apoE) is a 34 kDa glycoprotein with three distinct isoforms in the human population (apoE2, apoE3 and apoE4) known to play a major role in differentially influencing risk to, as well as outcome from, disease and injury in the centra... Read More about ApoE isoform-specific regulation of regeneration in the peripheral nervous system.

Valproate and Bone Loss: iTRAQ Proteomics Show that Valproate Reduces Collagens and Osteonectin in SMA Cells (2010)
Journal Article
Fuller, H. R., Man, N. T., Lam, L. T., Shamanin, V. A., Androphy, E. J., & Morris, G. E. (2010). Valproate and Bone Loss: iTRAQ Proteomics Show that Valproate Reduces Collagens and Osteonectin in SMA Cells. Journal of Proteome Research, 9(8), 4228-4233. https://doi.org/10.1021/pr1005263

Valproate is commonly used as an anticonvulsant and mood stabilizer, but its long-term side-effects can include bone loss. As a histone deacetylase (HDAC) inhibitor, valproate has also been considered for treatment of spinal muscular atrophy (SMA). U... Read More about Valproate and Bone Loss: iTRAQ Proteomics Show that Valproate Reduces Collagens and Osteonectin in SMA Cells.

Mesenchymal stem cell-conditioned medium accelerates skin wound healing: An in vitro study of fibroblast and keratinocyte scratch assays (2010)
Journal Article
Walter, M., Wright, K., Fuller, H., MacNeil, S., & Johnson, W. (2010). Mesenchymal stem cell-conditioned medium accelerates skin wound healing: An in vitro study of fibroblast and keratinocyte scratch assays. Experimental Cell Research, 316(7), 1271-1281. https://doi.org/10.1016/j.yexcr.2010.02.026

We have used in vitro scratch assays to examine the relative contribution of dermal fibroblasts and keratinocytes in the wound repair process and to test the influence of mesenchymal stem cell (MSC) secreted factors on both skin cell types. Scratch a... Read More about Mesenchymal stem cell-conditioned medium accelerates skin wound healing: An in vitro study of fibroblast and keratinocyte scratch assays.

The SMN Interactome Includes Myb-Binding Protein 1a (2009)
Journal Article
Fuller, H. R., Man, N. T., Lam, L. T., Thanh, L. T., Keough, R. A., Asperger, A., …Morris, G. E. (2010). The SMN Interactome Includes Myb-Binding Protein 1a. Journal of Proteome Research, 9(1), 556-563. https://doi.org/10.1021/pr900884g

Understanding networks of interacting proteins is a major goal in cell biology. The survival of motor neurons protein (SMN) interacts, directly or indirectly, with a large number of other proteins and reduced levels of SMN cause the inherited disorde... Read More about The SMN Interactome Includes Myb-Binding Protein 1a.

A two-site ELISA can quantify upregulation of SMN protein by drugs for spinal muscular atrophy (2008)
Journal Article
thi Man, N., Humphrey, E., Lam, L. T., Fuller, H. R., Lynch, T. A., Sewry, C. A., …Morris, G. E. (2008). A two-site ELISA can quantify upregulation of SMN protein by drugs for spinal muscular atrophy. Neurology, 71(22), 1757-1763. https://doi.org/10.1212/01.wnl.0000313038.34337.b1

Objectives: Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by loss of lower motor neurons during early or postnatal development. Severity is variable and is inversely related to the levels of survival of motor neurons... Read More about A two-site ELISA can quantify upregulation of SMN protein by drugs for spinal muscular atrophy.

Absence of gemin5 from SMN complexes in nuclear Cajal bodies (2007)
Journal Article
Hao, L. T., Fuller, H. R., Lam, L. T., Le, T. T., Burghes, A. H., & Morris, G. E. (2007). Absence of gemin5 from SMN complexes in nuclear Cajal bodies. BMC Cell Biology, 8(1), https://doi.org/10.1186/1471-2121-8-28

Background Spinal muscular atrophy is caused by reduced levels of the survival of motor neurons (SMN) protein. SMN is found in large complexes with Sm proteins and at least eight other proteins, including seven "gemins". These complexes are involved... Read More about Absence of gemin5 from SMN complexes in nuclear Cajal bodies.

IPSE/alpha-1: A major immunogenic component secreted from Schistosoma mansoni eggs (2006)
Journal Article
Schramm, G., Gronow, A., Knobloch, J., Wippersteg, V., Grevelding, C., Galle, J., …Doenhoff, M. (2006). IPSE/alpha-1: A major immunogenic component secreted from Schistosoma mansoni eggs. Molecular and Biochemical Parasitology, 147(1), 9-19. https://doi.org/10.1016/j.molbiopara.2006.01.003

During infection with Schistosoma mansoni the egg stage of this parasite modulates the initial T helper (Th1) response into a Th2 response. This suggests that schistosome eggs contain factors responsible for that effect. We have recently described a... Read More about IPSE/alpha-1: A major immunogenic component secreted from Schistosoma mansoni eggs.

An enzyme-linked immunosorbent assay (ELISA) for the major crustacean allergen, tropomyosin, in food (2006)
Journal Article
Fuller, H. R., Goodwin, P. R., & Morris, G. E. (2006). An enzyme-linked immunosorbent assay (ELISA) for the major crustacean allergen, tropomyosin, in food. Food and Agricultural Immunology, 17(1), 43-52. https://doi.org/10.1080/09540100600572651

Shellfish are a common cause of food reactions in hypersensitive individuals and are among the eight foods that account for over 90% of food allergies. At present, the only way to prevent these serious consequences of food allergies is to avoid the f... Read More about An enzyme-linked immunosorbent assay (ELISA) for the major crustacean allergen, tropomyosin, in food.

Stathmin is enriched in the developing corticospinal tract
Journal Article
Fuller, H. R., Slade, R., Jovanov-Milošević, N., Babić, M., Sedmak, G., Šimić, G., …Gates, M. A. (2015). Stathmin is enriched in the developing corticospinal tract. Molecular and Cellular Neuroscience, 12-21. https://doi.org/10.1016/j.mcn.2015.09.003

Understanding the intra- and extracellular proteins involved in the development of the corticospinal tract (CST) may offer insights into how the pathway could be regenerated following traumatic spinal cord injury. Currently, however, little is known... Read More about Stathmin is enriched in the developing corticospinal tract.