Katherine D. Mathews, MD
Research Profile

Research Interests:
My research career began with using genetic linkage, then a putative mouse model, to identify the gene causing facioscapulohumeral dystrophy.I closed my laboratory in 2000 due to increasing clinical and administrative demands, and my current academic efforts have been focused on improving the quality of care for patients with neuromuscular disease. I have maintained an active interest in the impact of molecular genetics on neuromuscular diseases (diagnosis, pathophysiology and treatment). I have served on NIH and CDC working groups to define the direction of research on neuromuscular disease in the future. I have become increasingly involved in collaborative clinical research efforts, many of which are laying a groundwork for clinical trials.

I am currently a co-PI (with Paul Romitti, PhD) on the Iowa MDSTARnet project, a CDC sponsored, multi-center Duchenne/Becker Muscular Dystrophy surveillance and epidemiology project. I am co-PI on one project of the University of Iowa’s NIH funded Wellstone Center, directed by Kevin Campbell, PhD and Steve Moore, MD, PhD. My project involves defining the phenotypes of patients with FRKP mutations and will extend nationwide. This clinical project was a key component of this successful NIH application. I am also the Iowa PI in the United Dystrophinopathy project; a genotype-phenotype study headed by Dr. Kevin Flanigan at the University of Utah and recently funded by the NIH.

Research Team:
Carrie Stephan, BSN
Karla Laubenthal, PT

Affiliations: Department of Pediatrics

Model System:
in vitro cell culture model, animal model, human trials, translational research, health services research

Recent Publications:

1. Moxley RT 3rd, Ashwal S, Pandya S, Connolly A, Florence J, Mathews K, Baumbach L, McDonald C, Sussman M, Wade C; Quality Standards Subcommittee of the American Academy of Neurology; Practice Committee of the Child Neurology Society. Practice parameter: corticosteroid treatment of Duchenne dystrophy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 11;64(1):13-20 Jan 2005
2. Max JE, Manes FF, Robertson BA, Mathews KD, Fox PT, Lancaster J. Prefrontal and executive attention network lesions and the development of attention-deficit/hyperactivity symptomatology. J Am Acad Child Adolesc Psychiatry. 44(5):443-50 May 2005.
3. Markham LW, Spicer RL, Khoury PR, Wong BL, Mathews KD, Cripe LH. Steroid Therapy and Cardiac Function in Duchenne Muscular Dystrophy. Pediatric Cardiology. 26(6):768-71 Nov-Dec 2005.
4. Ballester LY, Benson DW, Wong B, Law IH, Mathews KD, Vanoye CG, George AL Jr. Trafficking-competent and trafficking-defective KCNJ2 mutations in Andersen Syndrome. Hum Mutat. 27(4):388 Apr 2006.
5. Miller LA, Romitti P, Cunniff C, Druschel C, Mathews KD, Meaney J, Matthews D, Miller T, Andrews J, Fox D, Ciafaloni E, Pandya S, Montgomery A, Kenneson A, Zhen-Fang J. The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): Surveillance Methodology. Birth Defects Res A Clin Mol Teratol. 76(11):793-7Nov 2006.
6. Moore SA, Shilling CJ, Westra S, Wall C, Wicklund MP, Stolle C, Brown CA, Michele DE, Piccolo F, Winder T, Stence A, Barrese R, King N, King W, Florence J, Campbell KP, Fenichel GM, Stedman HH, Kissel JT, Griggs RC, Pandya S, Mathews KD, Pestronk A, Serrano C, Darvish D, Mendell JR. Limb-Girdle Muscular Dystrophy in the United States. J Neuropathol Exp Neurol 65(10):995-1003, Oct 2006.
7. Gomez C, Wilmot G, Perlman S, Mathews K, Subramony S, Ashizawa T, Tsou A, Myers L. Antioxidant Use in Friedreich Ataxia. J Neurological Sciences. Accepted for publication October 2007.