Significant Publications

Freeman SB, Allen EG, Oxford-Wright CL, Tinker SW, Druschel C, Hobbs CA, O'Leary LA, Romitti PA, Royle MH, Torfs CP, Sherman SL. The National Down Syndrome Project: design and implementation. Public Health Reports. 2007;122:62-72.

Bird TM, Hobbs CA, Cleves MA, Tilford JM, Robbins JM, National rates of birth defects among hospitalized newborns.[see comment]. Birth Defects Res. 2006;76:762-769.

Canfield MA, Honein MA, Yuskiv N, Xing J, Mai CT, Collins JS, Devine O, Petrini J, Ramadhani TA, Hobbs CA, Kirby RS. National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999-2001. Birth Defects Res. 2006;76:747-756..

Robbins JM, Bird TM, Tilford JM, Reading JA, Cleves MA, Aitken ME, Druschel CM, Hobbs CA. Reduction in newborns with discharge coding of in utero alcohol effects in the United States, 1993 to 2002. Arch Peds Adolesc Med. 2006;160:1224-1231.

Robbins JM, Tilford JM, Bird TM, Cleves MA, Reading JA, Hobbs CA. Hospitalizations of newborns with folate-sensitive birth defects before and after fortification of foods with folic acid. Pediatrics. 2006;118:906-915.

Robbins JM, Hopkins SE, Mosley BS, Casey PH, Cleves MA, Hobbs CA. Awareness and use of folic acid among women in the lower Mississippi Delta. J  Rural Health. 2006;22:196-203.

Zhao W, Mosley BS, Cleves MA, Melnyk S, James SJ, Hobbs CA. Neural tube defects and maternal biomarkers of folate, homocysteine, and glutathione metabolism. Birth Defects Res. 2006;76:230-236. 

Hobbs CA, James SJ, Parsian A, Krakowiak PA, Jernigan S, Greenhaw JJ, Lu Y, Cleves MA. Congenital heart defects and genetic variants in the methylenetetrahydroflate reductase gene. J Med Genet. 2006;43:162-166 .

Hobbs CA, Malik S, Zhao W, James SJ, Melnyk S, Cleves MA. Maternal homocysteine and congenital heart defects. J Am Coll Cardiol. 2006;47:683-685. 

Hobbs CA, James SJ, Jernigan S, Melnyk S, Lu Y, Malik S, Cleves MA. Congenital heart defects, maternal homocysteine, smoking, and the 677 C>T polymorphism in the methylenetetrahydrofolate reductase gene: evaluating gene-environment interactions. Am J Obstet Gynecol .2006;200:218-224.

Hobbs CA, Cleves MA, Melnyk S, Zhao W, James SJ. Congenital heart defects and abnormal maternal biomarkers of methionine and homocysteine metabolism. Am J Clin Nutr. 2005;81:147-153.

Hobbs CA, Cleves MA, Keith C, Ghaffar S, James  SJ. NKX2.5 and congenital heart defects: A population-based study. Am J Med Genet. 2005; 134A:223-225.

Hobbs CA, Cleves MA, Melnyk S, Zhao W, James SJ. Congenital heart defects and abnormal biomarkers of oxidative stress. Am J Clin Nutr.2005;82:598-604.

Cleves MA. Exploratory analysis of single nucleotide polymorphism (SNP) for quantitative traits. Stata Journal 2005;5:141-153.

Robbins JM, Cleves MA, Collins HB, Andrews N, Smith LN, Hobbs CA. Randomized trial of a physician – based intervention to increase women’s use of folic acid supplements. Am J Obstet Gynecol. 2005;192:1126-1132.

 Cleves MA, Hobbs CA. Collaborative strategies for unraveling the complexity of birth defects. J Matern Fetal Neonatal Med. 2004;15:35-38.

 Tilford JM, Cleves MA, Ghaffar S. Management of hypoplastic left heart syndrome. Pediatrics. 2004 113:431-432; author reply 431-432. 

 Cleves MA, Savell VH Jr, Raj S, Zhao W, Correa A, Werler MM, Hobbs CA; National Birth Defects Prevention Study. Maternal use of acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs), and muscular ventricular septal defects. Birth Defects Research Part A Clin Mol Teratol. 2004; 70:107-113. 

Cleves MA, Hobbs CA, Collins HB, Andrews N, Smith LN, Robbins JM. Folic acid use by women receiving routine gynecologic care. Obstet Gynecol. 2004;103:746-53.

Schwahn BC, Laryea MD, Chen Z, Melnyk S, Pogribny I, Garrow T, James SJ, Rozen R. Betaine rescue of an animal model with methylenetetrahydrofolate reductase deficiency. Biochem J. 2004 Jun 24;Pt. [Epub ahead of print]

James SJ. Maternal metabolic phenotype and risk of Down syndrome: beyond genetics. Am J Med Genet. 2004;127A:1-4.

Baric I, Fumic K, Glenn B, Cuk M, Schulze A, Finkelstein JD, James SJ, Mejaski-Bosnjak V, Pazanin L, Pogribny IP, Rados M, Sarnavka V, Scukanec-Spoljar M, Allen RH, Stabler S, Uzelac L, Vugrek O, Wagner C, Zeisel S, Mudd SH. S-adenosylhomocysteine hydrolase deficiency in a human: a genetic disorder of methionine metabolism. Proc Natl Acad Sci U S A. 2004;101:4234-4239.

Wyszynski DF, Baldwin CT, Cleves MA, Amirault Y, Nolan VG, Farrell JJ, Bisbee A, Kutlar A, Farrer LA, Steinberg MH. Polymorphisms near a chromosome 6q QTL area are associated with modulation of fetal hemoglobin levels in sickle cell anemia. Cell Mol Biol (Noisy-le-grand). 2004;50:23-33.

MacLennan NK, James SJ, Melnyk S, Piroozi A, Jernigan S, Hsu JL, Janke SM, Pham TD, Lane RH. Uteroplacental insufficiency alters DNA methylation, one-carbon metabolism, and histone acetylation in IUGR rats. Physiol Genomics. 2004;18:43-50.

Taub JW, Mundschau G, Ge Y, Poulik JM, Qureshi F, Jensen T, James SJ, Matherly LH, Wechsler J, Crispino JD. Prenatal origin of GATA1 mutations may be an initiating step in the development of megakaryocytic leukemia in Down syndrome. Blood. 2004;104:1588-1589.

Simmons CJ, Mosley BS, Fulton –Bond CA, Hobbs CA. Prevalence of birth defects in Arkansas: Is folic acid fortification making a difference? Birth Defects Res Part A Clin Mol Teratol. 2004;70:559-564.

Araneta MRG, Schlangen KM, Edmonds LD, Destiche DA, Merz RD, Hobbs CA, Flood TJ, Harris JA, Krishnamurti D, Gray GC. Prevalence of birth defects among infants of Gulf War veterans in Arkansas, Arizona, California, Georgia, Hawaii and Iowa, 1989-1993.  Birth Defects Research (Part A) 2003;67:246-260.

Cleves MA, Hobbs CA, Zhao W, Mosley BS. First-year survival of infants born with congenital heart defects in Arkansas (1993-1998): A survival analysis using registry data.  Birth Defects Research (Part A) 2003;67:662-668.

Hine RJ. Folic acid in congenital and chronic diseases: Nature and nurture. AGRO-food Industry Hi-Tech. 2003;14:33-37.

Yetman AT, Greenberg SB, Ghaffar S. Diaphragmatic hernia. Pediatric Cardiology 2003;24(3):307-8.

Tilford JM, Cleves MA, Ghaffar S, Chang RK, Chen A, Klitzner T. Management of Hypoplastic Left Heart Syndrome.  Pediatrics Nov 1, 2003; 112.

Innis SM, Davidson AGF, Chen A, Dyer R, Melnyk S, James SJ. Increased plasma homocysteine and S-adenosylhomocysteine and decrdased methionine is associated with altered phosphatidylcholine and phosphatidylethanolamine in cystic fibrosis. J Pediatr 2003; 143:351-356.

Carmel R, Melnyk S, James SJ. Cobalamin deficiency with and without neurologic abnormalities: Differences in homocysteine and methionine metabolism.  Blood 2003; 101:3301-3308.

Spiegelstein O, Lu  Le XC, Troen A, Selhub J, Melnyk S, James SJ, Finnell R. Effects of dietary folate intake and folate binding protein-1 (FOLBP1) on urinary speciation of arsenate in mice.  Toxicology Letters. 2003; 145:167-74.

Sung SS, Brassington AM, Krakowiak PA, Carey JC, Jorde LB, Bamshad M.  Mutations in TNNT3 cause multiple congenital contractures: a second locus for distal arthrogryposis type 2B. Am J Hum Genet. 2003 Jul;73:212-214.

Krakowiak PA, Wassif CA, Kratz L, Cozma D, Kovarova M, Harris G, Grinberg A, Yang Y, Hunter AG, Tsokos M, Kelley RI, Porter FD. Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency. Hum Mol Genet. 2003;12:1631-1641.

Cooper MK, Wassif CA, Krakowiak PA, Taipale J, Gong R, Kelley RI, Porter FD, Beachy PA. A defective response to Hedgehog signaling in disorders of cholesterol biosynthesis.  Nat Genet. 2003; 33:508-513.

Sung SS, Brassington AM, Grannatt K, Rutherford A, Whitby FG, Krakowiak PA, Jorde LB, Carey JC, Bamshad M.  Mutations in genes encoding fast-twitch contractile proteins cause distal arthrogryposis syndromes.  Am J Hum Genet. 2003;72:681-690.

James SJ, Pogribny IP, Pogribna M, Miller B, Jernigan S, Melnyk S. Mechanism of DNA damage, DNA hypomethylation, and tumor progression in the folate/methyl-deficient rat model of hepatocarcinogenesis. J Nutr. 2003;133:3740S-3747S.

 Hobbs CA, Cleves MA, Lauer RM, Burns TL, James SJ.   Preferential transmission of the MTHFR 677 T Allele to infants with Down Syndrome: Implications for a survival advantage.  Am J Med Genet 2002;113:9-14.

Rasmussen SA, Lammer EJ, Shaw GM, Finnell RH, McGehee, Jr., RE, Gallagher M, Romitti PA, Murray JC. Integration of DNA sample collection into a multi-site birth defects case-control study.   Teratology 2002;66:177-184.

Mosley BS, Simmons CJ, Cleves MA, Hobbs CA. Regional bias in birth defect prevalence rates for Arkansas: Influence of incomplete ascertainment along surveillance system borders. Teratology 2002;66:S36-S40. 

Hobbs CA. Cleves MA. Simmons CJ. Genetic epidemiology and congenital malformations: from the chromosome to the crib. Arch Peds Adolesct Med 2002;156:315-320.

James SJ, Hobbs CA. Chapter Title: "Folate deficiency and the molecular determinants of chromosome instability: Possible link to meiotic nondisjunction and Down syndrome." In: Massaro EJ and Rogers J, eds. Folate and human development. Humana Press, NJ 2002.

Al-Gazali LI, Melnyk S, Yi P, Pogribny IP, Pogribna M, Bakir M, Hamid ZA, Dawodu A, James SJ. Abnormal folate metabolism and genetic polymorphism of the folate pathway in a child with Down syndrome and neural tube defect. Am J Med Genet 2001;103:128-132.

Hassold TJ, Burrage LC, Chan ER, Judis LM, James SJ, Jacobs PA, Thomas NS. Maternal folate polymorphisms and the etiology of human nondisjunction. Am J Human Genet. 2001;69:434-439.

Hobbs CA, Hopkins SE, Simmons CJ. Sources of variability in birth defects prevalence rates. Teratology. 2001;64:S8-S13.

Pogribna M, Melnyk S, Pogribny IP, Yi P, Chango A, James SJ. Homocysteine metabolism in children with Down syndrome: In vitro modulation. Am J Human Genet. 2001;69:88-95.

Tilford JM, Robbins JM, Hobbs CA. Improving estimates of caregiver time cost and family impact associated with birth defects. Teratology. 2001;64:S37-S41.

Yadrick K, Horton J, Stuff J, McGee B, Bogle M, Davis L, Forrester J, Strickland E, Casey P, et al. Perceptions of community nutrition and health needs in the Lower Mississippi Delta: A key informant approach. J Nutr Educ. 2001;33:266-27.

Yoon PW, Rasmussen SA, Lynberg MC, Moore CA, Anderka M, Carmichael SL, Costa P, Druschel C, Hobbs CA, Romitti PA, Langlois P, Edmonds LD. The National Birth Defects Prevention Study. Public Health Reports. 2001;116(Supp 1):32-40.

Hobbs CA, Sherman SL, Yi P, Hopkins SE, Torfs CP, Hine RJ, Pogribna M, Rozen R, James SJ. Polymorphisms in genes involved in folate metabolism as maternal risk factors for Down syndrome. Am J Hum Genet. 2000;67:623-630.

James SJ, Pogribna M, Pogribny IP, Melnyk S, Hine RJ, Gibson JB, Yi P, Tafoya DL, Swenson DH, Wilson VL, Gaylor DW. Abnormal folate metabolism and mutation in the methylene tetrahydrofolate reductase gene may be maternal risk factors for Down syndrome. Am J Clin Nutr. 1999;70:495-501.