Steyn Group - Publications

Wong JPH, Blazev R, Ng YK, et al. Characterization of the skeletal muscle arginine methylome in health and disease reveals remodeling in amyotrophic lateral sclerosis. Faseb J 2024;38:e23647. https://www.ncbi.nlm.nih.gov/pubmed/38787599

Sajiir H, Keshvari S, Wong KY, et al. Liver and pancreatic-targeted interleukin-22 as a therapeutic for metabolic dysfunction-associated steatohepatitis. Nat Commun 2024;15:4528. PMC11137118: https://www.ncbi.nlm.nih.gov/pubmed/38811532

Jiang L, Tracey TJ, Gill MK, et al. Generation of human induced pluripotent stem cell lines from sporadic, sporadic frontotemporal dementia, familial SOD1, and familial C9orf72 amyotrophic lateral sclerosis (ALS) patients. Stem Cell Res 2024;78:103447. https://www.ncbi.nlm.nih.gov/pubmed/38796984

Howe SL, Holdom CJ, McCombe PA, et al. Associations of postprandial ghrelin, liver-expressed antimicrobial peptide 2 and leptin levels with body composition, disease progression and survival in patients with amyotrophic lateral sclerosis. Eur J Neurol 2024;31:e16052. PMC10840749: https://www.ncbi.nlm.nih.gov/pubmed/37658515

Holdom CJ, Janse van Mantgem MR, He J, et al. Variation in Resting Metabolic Rate Affects Identification of Metabolic Change in Geographically Distinct Cohorts of Patients With ALS. Neurology 2024;102:e208117. https://www.ncbi.nlm.nih.gov/pubmed/38350046

Choudhury C, Gill MK, McAleese CE, et al. The Arylamine N-Acetyltransferases as Therapeutic Targets in Metabolic Diseases Associated with Mitochondrial Dysfunction. Pharmacol Rev 2024;76:300-320. https://www.ncbi.nlm.nih.gov/pubmed/38351074

Shefner JM, Musaro A, Ngo ST, et al. Skeletal muscle in amyotrophic lateral sclerosis. Brain 2023;146:4425-4436. PMC10629757: https://www.ncbi.nlm.nih.gov/pubmed/37327376

Ludolph A, Dupuis L, Kasarskis E, Steyn F, Ngo S, McDermott C. Nutritional and metabolic factors in amyotrophic lateral sclerosis. Nat Rev Neurol 2023;19:511-524. https://www.ncbi.nlm.nih.gov/pubmed/37500993

Holdom CJ, van Unnik JWJ, van Eijk RPA, et al. Use of hip- versus wrist-based actigraphy for assessing functional decline and disease progression in patients with motor neuron disease. J Neurol 2023;270:2597-2605. PMC10129939: https://www.ncbi.nlm.nih.gov/pubmed/36740646

Guillou A, Kemkem Y, Lafont C, et al. TSH Pulses Finely Tune Thyroid Hormone Release and TSH Receptor Transduction. Endocrinology 2023;165. PMC10666572: https://www.ncbi.nlm.nih.gov/pubmed/37934802

Chang J, Shaw TB, Holdom CJ, et al. Lower hypothalamic volume with lower body mass index is associated with shorter survival in patients with amyotrophic lateral sclerosis. Eur J Neurol 2023;30:57-68. PMC10099625: https://www.ncbi.nlm.nih.gov/pubmed/36214080

Berlowitz DJ, Mathers S, Hutchinson K, et al. The complexity of multidisciplinary respiratory care in amyotrophic lateral sclerosis. Breathe (Sheff) 2023;19:220269. PMC10567075: https://www.ncbi.nlm.nih.gov/pubmed/37830099

Scaricamazza S, Salvatori I, Amadio S, et al. Repurposing of Trimetazidine for amyotrophic lateral sclerosis: A study in SOD1(G93A) mice. Br J Pharmacol 2022;179:1732-1752. PMC9305494: https://www.ncbi.nlm.nih.gov/pubmed/34783031

Restuadi R, Steyn FJ, Kabashi E, et al. Functional characterisation of the amyotrophic lateral sclerosis risk locus GPX3/TNIP1. Genome Med 2022;14:7. PMC8767698: https://www.ncbi.nlm.nih.gov/pubmed/35042540

Restuadi R, Garton FC, Benyamin B, et al. Polygenic risk score analysis for amyotrophic lateral sclerosis leveraging cognitive performance, educational attainment and schizophrenia. Eur J Hum Genet 2022;30:532-539. PMC9090723: https://www.ncbi.nlm.nih.gov/pubmed/33907316

McGill RB, Steyn FJ, Ngo ST, et al. Monocyte CD14 and HLA-DR expression increases with disease duration and severity in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2022;23:430-437. https://www.ncbi.nlm.nih.gov/pubmed/34396845

Hop PJ, Zwamborn RAJ, Hannon E, et al. Genome-wide study of DNA methylation shows alterations in metabolic, inflammatory, and cholesterol pathways in ALS. Sci Transl Med 2022;14:eabj0264. PMC10040186: https://www.ncbi.nlm.nih.gov/pubmed/35196023

Holdom CJ, Steyn FJ, Henderson RD, McCombe PA, Rogers ML, Ngo ST. Biofluid Biomarkers of Amyotrophic Lateral Sclerosis. Neurodegenerative Diseases Biomarkers 2022;173:263-306. <Go to ISI>://WOS:000868553200013

Holdom CJ, Ngo ST, McCombe PA, Henderson RD, Steyn FJ. Low plasma hyaluronan is associated with faster functional decline in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2022;23:42-48. https://www.ncbi.nlm.nih.gov/pubmed/34569358

Helleman J, Johnson B, Holdom C, et al. Patient perspectives on digital healthcare technology in care and clinical trials for motor neuron disease: an international survey. J Neurol 2022;269:6003-6013. PMC9294855: https://www.ncbi.nlm.nih.gov/pubmed/35849154

Ding Q, Kesavan K, Lee KM, et al. Impaired signaling for neuromuscular synaptic maintenance is a feature of Motor Neuron Disease. Acta Neuropathol Commun 2022;10:61. PMC9040261: https://www.ncbi.nlm.nih.gov/pubmed/35468848

van Rheenen W, van der Spek RAA, Bakker MK, et al. Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology. Nat Genet 2021;53:1636-1648. PMC8648564: https://www.ncbi.nlm.nih.gov/pubmed/34873335

van Eijk RPA, Beelen A, Kruitwagen ET, et al. A Road Map for Remote Digital Health Technology for Motor Neuron Disease. J Med Internet Res 2021;23:e28766. PMC8495582: https://www.ncbi.nlm.nih.gov/pubmed/34550089

Tracey TJ, Kirk SE, Steyn FJ, Ngo ST. The role of lipids in the central nervous system and their pathological implications in amyotrophic lateral sclerosis. Semin Cell Dev Biol 2021;112:69-81. https://www.ncbi.nlm.nih.gov/pubmed/32962914

Toufaily C, Fortin J, Alonso CA, et al. Addition of a carboxy-terminal tail to the normally tailless gonadotropin-releasing hormone receptor impairs fertility in female mice. Elife 2021;10. PMC8741216: https://www.ncbi.nlm.nih.gov/pubmed/34939930

Tefera TW, Steyn FJ, Ngo ST, Borges K. CNS glucose metabolism in Amyotrophic Lateral Sclerosis: a therapeutic target? Cell Biosci 2021;11:14. PMC7798275: https://www.ncbi.nlm.nih.gov/pubmed/33431046

Sidhom S, Schneider A, Fang Y, et al. 17alpha-Estradiol Modulates IGF1 and Hepatic Gene Expression in a Sex-Specific Manner. J Gerontol A Biol Sci Med Sci 2021;76:778-785. PMC8087270: https://www.ncbi.nlm.nih.gov/pubmed/32857104

Ongaro L, Alonso CAI, Zhou X, et al. Development of a Highly Sensitive ELISA for Measurement of FSH in Serum, Plasma, and Whole Blood in Mice. Endocrinology 2021;162. PMC7894055: https://www.ncbi.nlm.nih.gov/pubmed/33475143

Ngo ST, Wang H, Henderson RD, Bowers C, Steyn FJ. Ghrelin as a treatment for amyotrophic lateral sclerosis. J Neuroendocrinol 2021;33:e12938. https://www.ncbi.nlm.nih.gov/pubmed/33512025

Nabais MF, Laws SM, Lin T, et al. Meta-analysis of genome-wide DNA methylation identifies shared associations across neurodegenerative disorders. Genome Biol 2021;22:90. PMC8004462: https://www.ncbi.nlm.nih.gov/pubmed/33771206

Holdom CJ, Janse van Mantgem MR, van Eijk RPA, et al. Venous creatinine as a biomarker for loss of fat-free mass and disease progression in patients with amyotrophic lateral sclerosis. Eur J Neurol 2021;28:3615-3625. https://www.ncbi.nlm.nih.gov/pubmed/34216521

Blackmore DG, Steyn FJ, Carlisle A, et al. An exercise "sweet spot" reverses cognitive deficits of aging by growth-hormone-induced neurogenesis. iScience 2021;24:103275. PMC8567379: https://www.ncbi.nlm.nih.gov/pubmed/34761193

Ahmed RM, Steyn F, Dupuis L. Hypothalamus and weight loss in amyotrophic lateral sclerosis. Handb Clin Neurol 2021;180:327-338. https://www.ncbi.nlm.nih.gov/pubmed/34225938

Steyn FJ, et al. Prognostic value of weight loss in patients with amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2020;91:813.

Steyn FJ, et al. Altered skeletal muscle glucose-fatty acid flux in amyotrophic lateral sclerosis. Brain Commun 2020;2:fcaa154.

Steyn FJ, et al. Altered skeletal muscle glucose-fatty acid flux in amyotrophic lateral sclerosis. bioRxiv 2020;2020.04.03.021238.

Sidhom S, et al. 17alpha-estradiol modulates IGF1 and hepatic gene expression in a sex-specific manner. J Gerontol A Biol Sci Med Sci 2020.

Scaricamazza S, et al. Skeletal-Muscle Metabolic Reprogramming in ALS-SOD1(G93A) Mice Predates Disease Onset and Is A Promising Therapeutic Target. iScience 2020;23:101087.

Ngo ST, et al. Progression and survival of patients with motor neuron disease relative to their fecal microbiota. Amyotroph Lateral Scler Frontotemporal Degener 2020;21:549-562.

Nabais MF, et al. Significant out-of-sample classification from methylation profile scoring for amyotrophic lateral sclerosis. Npj Genom Med 2020;5:10.

McGill RB, et al. Monocytes and neutrophils are associated with clinical features in amyotrophic lateral sclerosis. Brain Commun 2020;2:fcaa013.

Joustra SD, et al. Response to Letter to the Editor: "IGSF1 Deficiency Results in Human and Murine Somatotrope Neurosecretory Hyperfunction". J Clin Endocrinol Metab 2020;105.

Joustra SD, et al. IGSF1 Deficiency Results in Human and Murine Somatotrope Neurosecretory Hyperfunction. J Clin Endocr Metab 2020;105:E70-E84.

Iacoangeli A, et al. Genome-wide Meta-analysis Finds the ACSL5-ZDHHC6 Locus Is Associated with ALS and Links Weight Loss to the Disease Genetics. Cell Rep 2020;33:108323.

Ngo ST, et al. Loss of appetite is associated with a loss of weight and fat mass in patients with amyotrophic lateral sclerosis. Amyotroph Lat Scl Fr 2019;20:497-505.

Nabizadeh JA, et al. C5a receptors C5aR1 and C5aR2 mediate opposing pathologies in a mouse model of melanoma. Faseb J 2019;33:11060-11071.

Nabizadeh J, et al. Complement C5a receptors, C5aR1 and C5aR2, mediate opposing pathologies in a mouse model of melanoma. Eur J Immunol 2019;49:174-174.

McCombe PA, et al. Patient with ALS with a novel TBK1 mutation, widespread brain involvement, behaviour changes and metabolic dysfunction. J Neurol Neurosur Ps 2019;90:952-954.

McCombe PA, et al. Gut microbiota in ALS: possible role in pathogenesis? Expert Rev Neurother 2019;19:785-805.

Kirk SE, et al. Biomarkers of Metabolism in Amyotrophic Lateral Sclerosis. Front Neurol 2019;10.

Tracey TJ, et al. Neuronal Lipid Metabolism: Multiple Pathways Driving Functional Outcomes in Health and Disease. Front Mol Neurosci 2018;11.

Steyn FJ, et al. 17 alpha-estradiol acts through hypothalamic pro-opiomelanocortin expressing neurons to reduce feeding behavior. Aging Cell 2018;17.

Steyn FJ, et al. Hypermetabolism in ALS is associated with greater functional decline and shorter survival. J Neurol Neurosur Ps 2018;89:1016-1023.

Varamini P, et al. Development of New Gonadotropin-Releasing Hormone-Modified Dendrimer Platforms with Direct Antiproliferative and Gonadotropin Releasing Activity. J Med Chem 2017;60:8309-8320.

Varamini P, et al. New gonadotropin-releasing hormone glycolipids with direct antiproliferative activity and gonadotropin-releasing potency. Int J Pharm 2017;521:327-336.

Stout MB, et al. 17 alpha-Estradiol Alleviates Age-related Metabolic and Inflammatory Dysfunction in Male Mice Without Inducing Feminization. J Gerontol a-Biol 2017;72:3-15.

Steyn FJ, et al. Comparison of Faecal Microbe Diversity between Motor Neurone Disease (Mnd) and Control Participants. J Neurol Neurosur Ps 2017;88.

Steyn FJ, et al. Endocrine rhythms of growth hormone release: Insights from animal studies. Best Pract Res Cl En 2017;31:521-533.

Steyn FJ. Growth Hormone Releasing Hormone.  Encyclopedia of Signaling Molecules, 2nd edition: Springer, 2017.

Spiers JG, et al. Noninvasive assessment of altered activity following restraint in mice using an automated physiological monitoring system. Stress 2017;20:59-67.

Ngo ST, et al. Exploring targets and therapies for amyotrophic lateral sclerosis: current insights into dietary interventions. Degener Neurol Neuro 2017;7:95-108.

Kirk SE, et al. Restraint stress increases prolactin-mediated phosphorylation of signal transducer and activator of transcription 5 in the hypothalamus and adrenal cortex in the male mouse. J Neuroendocrinol 2017;29.

Ioannides ZA, et al. Predictions of resting energy expenditure in amyotrophic lateral sclerosis are greatly impacted by reductions in fat free mass. Cogent Medicine 2017;4:1343000.

Ioannides ZA, et al. Anthropometric measures are not accurate predictors of fat mass in ALS. Amyotroph Lat Scl Fr 2017;18:486-491.

Ioannides ZA, et al. The Risk of Overestimating Fatness in Motor Neurone Disease: Longitudinal Assessments of Body Composition. J Neurol Neurosur Ps 2017;88.

Ioannides ZA, et al. Hypermetabolism in Motor Neurone Disease Is Associated with a Greater Functional Decline but Not Weight Loss. J Neurol Neurosur Ps 2017;88.

Wan Y, et al. The refinement of luteinizing hormone (LH) output during pubertal maturation is associated with the recruitment of follicle in adult female mice. Clin Endocrinol 2016;84:2-3.

Tuong ZK, et al. Transgenic Adipose-specific Expression of the Nuclear Receptor RORalpha Drives a Striking Shift in Fat Distribution and Impairs Glycemic Control. EBioMedicine 2016;11:101-117.

Tan HY, et al. Hyperphagia in male melanocortin 4 receptor deficient mice promotes growth independently of growth hormone. J Physiol-London 2016;594:7309-7326.

Steyn FJ, et al. Neuroendocrine Regulation of Growth Hormone Secretion. Compr Physiol 2016;6:687-735.

Steyn F. Regulation of pulsatile growth hormone secretion: lessons from the mouse. Clin Endocrinol 2016;84:16-17.

Nabizadeh JA, et al. The Complement C3a Receptor Contributes to Melanoma Tumorigenesis by Inhibiting Neutrophil and CD4(+) T Cell Responses. J Immunol 2016;196:4783-4792.

Nabizadeh J, et al. The complement C3a receptor contributes to melanoma tumourigenesis by regulating neutrophil and CD4(+) T cell responses. Eur J Immunol 2016;46:510-510.

Li R, et al. Development of a high-throughput method for real-time assessment of cellular metabolism in intact long skeletal muscle fibre bundles. J Physiol-London 2016;594:7197-7213.

Ioannides ZA, et al. Altered Metabolic Homeostasis in Amyotrophic Lateral Sclerosis: Mechanisms of Energy Imbalance and Contribution to Disease Progression. Neurodegener Dis 2016;16:382-397.

Czieselsky K, et al. Pulse and Surge Profiles of Luteinizing Hormone Secretion in the Mouse. Endocrinology 2016;157:4794-4802.