External References

The Role of NF-κB in DMD:

Fiorillo AA, Heier CR, Novak JS, Tully CB, Brown KJ, Uaesoontrachoon K, Vila MC, Ngheim PP, Bello L, Kornegay JN, Angelini C, Partridge TA, Nagaraju K, Hoffman EP. TNF-a-Induced microRNAs Control Dystrophin Expression in Becker Muscular Dystrophy. Cell Rep. 2015 Sep 8;12(10):1678-90.

Kumar A., Boriek AM. Mechanical stress activates the nuclear factor-kappaB pathway in skeletal muscle fibers: a possible role in Duchenne muscular dystrophy. FASEB J 2003 Mar; 17(3):17: 386-96.

Peterson JM, Bakkar N, Guttridge, DC. NF-kB signaling in skeletal muscle health and disease. Curr Top Dev Biol. 2011; 96: 85-119.

Porter JD, Merriam AP, Leahy P, Gong B, Khanna S. Dissection of temporal gene expression signatures of affected and spared muscle groups in dystrophin-deficient (mdx) mice. Hum Molecular Genetics 2003 Aug; 12(15):1813-1821.

MRI as an Objective Measure in DMD:

Akima H, Lott D, Senesac C, Deol J, Germain S, Arpan I, Bendixen R, Sweeney HL, Walter G, Vandenborne K. Relationships of thigh muscle contractile and non-contractile tissue with function, strength, and age in boys with Duchenne muscular dystrophy. Neuromuscular Discord. 2012 Jan: 22(1): 16-25.

Arpan I, Willcocks RJ, Forbes SC, Finkel RS, Lott DJ, Rooney WD, Triplett WT, Senesac CR, Daniels MJ, Byrne BJ, Finager RL, Russman BS, Wang DJ, Tennekoon GI, Walter GA, Sweeney HI, Vandenborne K. Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS. Neurology 2014 Sep; 83(11): 974-80.

Forbes SC, Walter GA, Rooney WD, Wang D, DeVos S, Pollaro J, Triplett W, Lott DJ, Willcocks RJ, Senesac C, Daniels MJ, Byrne BJ, Russman B, Finkel RS, Meyer JS, Sweeney HL, Vandenborne K. Skeletal Muscles of Ambulant Children with Duchenne Muscular Dystrophy: Validation of Multicenter Study of Evaluation with MR Imaging and MR Spectroscopy. Radiology. 2013 Oct;269(1):198-207.

Willcocks RJ, Arpan IA, Forbes SC, Lott DJ, Senesac CR, Senesac E, Deol J, Triplett WT, Baligand C, Daniels MJ, Sweeney HL, Walter GA, Vandenborne K. Longitudinal measurements of MRI-T2 in boys with Duchenne muscular dystrophy: effects of age and disease progression. Neuromuscul Disord. 2014 May; 24(5):393-401.

Willcocks RJ, Rooney WD, Triplett WT, Forbes SC, Lott DJ, Senesac CR, Daniels MJ, Wang DJ, Harrington AT, Tennekoon GI, Russman BS, Finanger EL, Byrne BJ, Finkel RS, Walter GA, Sweeney HL, Vandenborne K. Multicenter prospective longitudinal study of magnetic resonance biomarkers in a large Duchenne Muscular Dystrophy cohort. Ann Neurol. 2016 Apr;79(4):535-47.

Frakes AE, Ferraiulo L, Haidet-Phillips AM, Schmelzer L, Braun L, Miranda CJ, Ladner KJ, Bevan AK, Foust KD, Godbout JP, Popovich PG, Guttridge DC, Kaspar BK. Microglia induce motor neuron death via the classical NF-kB pathway in amyotrophic lateral sclerosis. Neuron 2014 81(5): 1009-23.

Johnson DA, Johnson JA. Nrf2—a therapeutic target for the treatment of neurodegenerative diseases. Free Radic Biol Med 2015 88(PtB): 253-67.

Shen Y, McMackin M, Shan Y, Raetz A, David S, Cortopassi G. Frataxin deficiency promotes excess microglial DNA damage and inflammation that is rescued by PJ34. PLoS ONE 2016 11(3).

Paupe V, Dassa EP, Goncalves S, Auchere F, Lonn M, Holmgren A, Rustin P. Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia. PLoS ONE 2009 4(1).

Armstrong JS, Khdour O, Hecht SM. Does oxidative stress contribute to the pathology of Friedreich’s ataxia? A radical question. FASEB J 2010 24(7): 2152-63.

Esposito S, Tosco A, Villela V, Raia V, Kroemer G, Maiuri L. Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis. Molecular and Cellular Pediatrics. 2016 December; 3(1):13.

Junkins R, McCormick C, Lin T. The emerging potential of autophagy-based therapies in the treatment of cystic fibrosis lung infections. Landes Bioscience. 2014 March; 10:3, 538-547.

Nakahira K, Pabon Porras M, Choi A. Autophagy in Pulmonary Diseases. American Journal of Respiratory and Critical Care Medicine. 2016 November;  194(10):1196-1207.

Ryter S, Choi A. Autophagy in lung disease pathogenesis and therapeutics. Redox Biology. 2015 January; 4:215-25.

The Role of SREBP in NASH

Shao W, Espenshade PJ. Expanding roles for SREBP in metabolism. Cell Metab. 2012 Oct; 16(4):414-19.

Watanabe M, Uesugi, M. Small-molecule inhibitors of SREBP activation – potential for new treatment of metabolic disorders. Med Chem Commun. 2013; 4: 1422-1433.