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Publications supported by the Center for Pediatric Research can be found here.

 

2018

Modeling rare diseases with induced pluripotent stem cell technology.

Anderson RH, Francis KR.
Molecular and cellular probes. 2018;
PMID:29307697

 

Self-Tranducible Bimodal PDX1-FOXP3 Protein Lifts Insulin Secretion and Curbs Autoimmunity, Boosting Tregs in Type 1 Diabetic Mice.

Amatya C, Radichev IA, Ellefson J, Williams M, Savinov AY
Molecular Therapy. 2018. 3;26(1):184-198
PMID:28988715 PMCID:PMC5762970

 

2017

Lack of specificity of antibodies raised against CLN3, the lysosomal/endosomal transmembrane protein mutated in juvenile Batten disease.

Nelson T, Pearce DA, Kovács AD.
Bioscience reports. 2017; 37(6).
PMID: 29089465 PMCID:PMC5700270

 

The Type 1 Diabetes-Resistance Locus Idd22 Controls Trafficking of Autoreactive CTLs into the Pancreatic Islets of NOD Mice.

Whitener RL, Gallo Knight L, Li J, Knapp S, Zhang S, Annamalai M, Pliner VM, Fu D, Radichev I, Amatya C, Savinov A, Yurdagul A Jr, Yuan S, Glawe J, Kevil CG, Chen J, Stimpson SE, Mathews CE
Journal of Immunology. 2017. 15;199(12):3991-4000
PMID:29109122 PMCID:PMC5716886

 

Nonmuscle myosin 2 proteins encoded by Myh9, Myh10, and Myh14 are uniquely distributed in the tubular segments of murine kidney.

Otterpohl KL, Hart RG, Evans C, Surendran K, Chandrasekar I.
Physiological reports. 2017; 5(23).
PMID: 29208685  PMCID: PMC5727274

 

Immunohistochemical Markers for Prospective Studies in Neurofibromatosis-1 Porcine Models

Meyerholz DK, Ofori-Amanfo GK, Leidinger MR, Goeken JA, Khanna R, Sieren JC, Darbro BW, Quelle DE, Weimer JM
Journal of Histochemistry and Cytochemistry. 2017. 65(10):607-618
PMID:28846462 PMCID:PMC5624366

 

Placental lipid processing in response to a maternal high-fat diet and diabetes in rats.

Louwagie EJ, Larsen TD, Wachal AL, Baack ML.
Pediatric research. 2017; 
PMID:  29166372

 

Overexpression of tissue-nonspecific alkaline phosphatase (TNAP) in endothelial cells accelerates coronary artery disease in a mouse model of familial hypercholesterolemia

Romanelli F, Corbo A, Salehi M, Yadav MC, Salman S, Petrosian D, Rashidbaigi OJ, Chait J, Kuruvilla J, Plmmer M, Radichev I, Margulies KB, Gerdes AM, Pinkerton AB, Millán JL, Savinov AY, Savinova OV
PLoS One. 2017. 12;12(10):e0186426
PMID:29023576 PMCID:PMC5638543

 

4SC-202 as a Potential Treatment for the Pediatric Brain Tumor Medulloblastoma.

Messerli SM, Hoffman MM, Gnimpieba EZ, Kohlhof H, Bhardwaj RD.
Brain sciences. 2017; 7(11).
PMID: 29099739  PMCID: PMC5704154

 

Pharmacological Effects on Ceroid Lipofuscin and Neuronal Structure in Cln3 ?ex7/8 Mouse Brain Cultures

Brenneman DE, Pearce DA, Kovács AD, DeFrees S
Journal of Molecular Neuroscience. 2017. 63(1):100-114
PMID:28812237

 

Extenuating Circumstances: Data Sharing: Leading Data-Driven Population Health

Griese ER, Hsu BS, Pearce DA
South Dakota Medicine. 2017. 70(8):374-375
PMID:28813747

 

Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity

Cárcel-Trullols J, Kovács AD, Pearce DA
Journal of Cellular Biochemistry. 2017. 118(11):3883-3890
PMID: 28390177 PMCID:PMC5603378

 

Genetic ataxia telangiectasia porcine model phenocopies the multisystemic features of the human disease.

Beraldi R, Meyerholz DK, Savinov A, Kovács AD, Weimer JM, Dykstra JA, Geraets RD, Pearce DA.
Biochimica et biophysica acta. 2017; 1863(11):2862-2870. NIHMSID: NIHMS913123
PMID:28746835 PMCID: PMC5687068

 

Molecular genetics of osteosarcoma.

Rickel K, Fang F, Tao J.
Bone. 2017; 102:69-79. NIHMSID: NIHMS824627
PMID: 27760307 PMCID: PMC5393957

 

VRK2A is an A-type lamin-dependent nuclear envelope kinase that phosphorylates BAF.

Birendra Kc, May DG, Benson BV, Kim DI, Shivega WG, Ali MH, Faustino RS, Campos AR, Roux KJ.
Molecular biology of the cell. 2017; 28(17):2241-2250.
PMID:28637768 PMCID:PMC5555652

 

ATOH1 Promotes Leptomeningeal Dissemination and Metastasis of Sonic Hedgehog Subgroup Medulloblastomas

Grausam KB, Dooyema SDR, Bihannic L, Premathilake H, Morrissy AS, Forget A, Schaefer AM, Gundelach JG, Macura S, Maher DM, Wang X, Heglin AH, Ge X, Zeng E, Puget S, Chandrasekar I, Surendran K, Bram RJ, Schüller U, Talyor MD, Ayrault O, Zhao H
Cancer Research. 2017. 15;77(14):3766-3777
PMID:28490517 PMCID:PMC5512702

 

Therapeutic Targeting of PTK7 is Cytotoxic in Atypical Teratoid Rhabdoid Tumors.

Messerli SM, Hoffman MM, Gnimpieba EZ, Bhardwaj RD.
Molecular cancer research : MCR. 2017; 15(8):973-983. NIHMSID: NIHMS871849
PMID:28442586 PMCID:PMC5594561

 

ATOH1 Promotes Leptomeningeal Dissemination and Metastasis of Sonic Hedgehog Subgroup Medulloblastomas.

Grausam KB, Dooyema SDR, Bihannic L, Premathilake H, Morrissy AS, Forget A, Schaefer AM, Gundelach JH, Macura S, Maher DM, Wang X, Heglin AH, Ge X, Zeng E, Puget S, Chandrasekar I, Surendran K, Bram RJ, Schüller U, Talyor MD, Ayrault O, Zhao H.
Cancer research. 2017; 77(14):3766-3777. NIHMSID: NIHMS875727
PMID:28490517 PMCID:PMC5512702

 

Intact piRNA pathway prevents L1 mobilization in male meiosis.

Newkirk SJ, Lee S, Grandi FC, Gaysinskaya V, Rosser JM, Vanden Berg N, Hogarth CA, Marchetto MCN, Muotri AR, Griswold MD, Ye P, Bortvin A, Gage FH, Boeke JD, An W.
Proceedings of the National Academy of Sciences of the United States of America. 2017; 114(28):E5635-E5644.
PMID:28630288 PMCID:PMC5514719

 

A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies

Geraets RD, Landin LM, Cain JT, Parker CM, Beraldi R, Kovács AD, Weimer JM, Pearce DA
PLoS One. 2017. 2;12(5):e0176526
PMID:28464005 PMCID:PMD5413059

 

MicroRNA miR-23a cluster promotes osteocyte differentiation by regulating TGF-β signaling in osetoblasts

Zeng HC, Bae Y, Dawson BC, Chen Y, Bertin T, Munivez E, Campeau PM, Tao J, Chen R, Lee BH
Nature Communications. 2017. 11;8:15000
PMID: 28397831 PMCID:PMC5394267

Prostaglandin E2 Indicates Therapeutic Efficacy of Mesenchymal Stem Cells in Experimental Traumatic Brain Injury

Kota DJ, Prabhakara KS, Toledano-Furman N, Bhattarai D, Chen Q, DiCarlo B, Smith P, Triolo F, Wenzel PL, Cos CS Jr, Olson SD
Stem Cells. 2017. 35(5):1416-1430
PMID:28233428

 

Prenatal Exposure to a Maternal High-Fat Diet Affects Histone Modification of Cardiometabolic Genes in Newborn Rats

Upadhyaya B, Larsen T, Barwari S, Louwagie EJ, Baack ML, Dey M
Nutrients. 2017. 20;9(4)
PMID:28425976  PMCID:PMC5409746

 

Nonsense pathogenic variants in exon 1 of PHOX2B lead to translational reinitiation in congenital central hypoventilation syndrome.

Cain JT, Kim DI, Quast M, Shivega WG, Patrick RJ, Moser C, Reuter S, Perez M, Myers A, Weimer JM, Roux KJ, Landsverk M.
American journal of medical genetics. Part A. 2017; 173(5):1200-1207. NIHMSID: NIHMS844586
PMID:28371199 PMCID:PMC5397359

 

Detailed Clinical Phenotype and Molecular Genetic Findings in CLN3-Associated Isolated Retinal Degeneration

Ku CA, Hull S, Arno G, Vincent A, Carss K, Kayton R, Weeks D, Anderson FW, Geraets R, Parker C, Pearce DA Michaleides M, MacLaren RE, Robson AG, Holder GE, Heon E, Raymond FL, Mooe AT, Webster AR, Pennesi ME
JAMA Opthalmology. 2017. 1;135(7):749-760
PMID:28542676 PMCID:PMC5710208

 

Identification and Characterization of microRNAs Associated With Human β-Cell Loss in a Mouse Model

Roat R, Hossain MM, Christopherson J, Free C, Jain S, Guay C, Regazzi R, Guo Z
American Journal of Transplantation. 2017. 17(4):992-1007
PMID: 27700000

 

Myt1l safeguards neuronal identity by actively repressing many non-neuronal fates

Mall M, Kareta MS, Chanda S, Ahlenius H, Perotti N, Zhou B, Grieder SD, Ge X, Drake S, Euong Ang C, Walker BM, Vierbuchen T, Fuentes DR, Brennecke P, Nitta KR, Jolma A, Steinmetz LM, Taipale J, Südhof TC, Wernig M
Nature. 2017. 13;544(7649):245-249
PMID:28379941

 

Concise Review: Fat and Furious: Harnessing the Full Potential of Adipose-Derived Stromal Vascular Fraction.

Dykstra JA, Facile T, Patrick RJ, Francis KR, Milanovich S, Weimer JM, Kota DJ.
Stem cells translational medicine. 2017; 6(4):1096-1108. NIHMSID: NIHMS830305
PMID:28186685 PMCID:PMC5388064

 

Elf5 is a principal cell lineage specific transcription factor in the kidney that contributes to Aqp2 and Avpr2 gene expression.

Grassmeyer J, Mukherjee M, deRiso J, Hettinger C, Bailey M, Sinha S, Visvader JE, Zhao H, Fogarty E, Surendran K.
Developmental biology. 2017; 424(1):77-89. NIHMSID: NIHMS854169
PMID:28215940 PMCID:PMC5382981

 

Creating a Reliable, Cost-Effective ELISA Simulation

Olson J, Amolins M, Vitiello P
The American Biology Teacher. 2017. 79(4):301-304
PMID:29033458 PMCID:PMC5640158

 

The cohesion subunit Rad21 is a negative regulator of hematopoietic self-renewal through epigenetic repression of Hoxa7 and Hoxa9.

Fisher JB, Peterson J, Reimer M, Stelloh C, Pulakanti K, Gerbec ZJ, Abel AM, Strouse JM, Strouse C, McNulty M, Malarkannan S, Crispino JD, Milanovich, S, Rao S
Leukemia. 2017. 31(3):712-719
PMID:27554164 PMCID:PMC5332284

 

Three dimensional electrospun PCL/PLA blend nanofibrous scaffolds with significantly improved stem cells osteogenic differentiation and cranial bone formation.

Yao Q, Cosme JG, Xu T, Miszuk JM, Picciani PH, Fong H, Sun H.
Biomaterials. 2017; 115:115-127. NIHMSID: NIHMS832000
PMID:27886552 PMCID:PMC5181114

 

Detoxification of Mitochondrial Oxidants and Apoptotic Signaling Are Facilitated by Thioredoxin-2 and Peroxiredoxin-3 during Hyperoxic Injury.

Forred BJ, Daugaard DR, Titus BK, Wood RR, Floen MJ, Booze ML, Vitiello PF.
PloS one. 2017; 12(1):e0168777.
PMID:28045936 PMCID:PMC5207683

 

Decreased sensitivity of palmitoyl protein thoesterase 1-deficient neurons to chemical anoxia

Meyer M, Kovács AD, Pearce DA
Metabolic Brain Disease. 2017. 32(1):275-279
PMID:27722792 PMCID:PMC5335868

 

BBP-Functionalized Biomimetic Nanofibrous Scaffold Can Capture BMP2 and Promote Osteogenic Differentiation.

Yao Q, Sandhurst ES, Liu Y, Sun H.
Journal of materials chemistry. B. 2017; 5(26):5196-5205
PMID:29250330 PMCID:PMC5730084

 

Breast milk DHA levels may increase after informing women: a community based cohort study from South Dakota USA.

Juber BA, Jackson KH, Johnson KB, Harris WS, Baack ML
International Breastfeeding Journal. 2017; 12:7
PMID:28149321 PMCID:PMC5273852

 

 

2016

The mammalian LINC complex regulates genome transcriptional responses to substrate rigidity.

Alam SG, Zhang Q, Prasad N, Li Y, Chamala S, Kuchibhotla R, Kc B, Aggarwal V, Shrestha S, Jones AL, Levy SE, Roux KJ, Nickerson JA, Lele TP.
Scientific reports. 2016; 6:38063.
PMID:27905489 PMCID:PMC5131312

 

Hypoxia-Mimicking Nanofibrous Scaffolds Promote Endogenous Bone Regeneration.

Yao Q, Liu Y, Tao J, Baumgarten KM, Sun H.
ACS applied materials & interfaces. 2016; 8(47):32450-32459. NIHMSID: NIHMS828748
PMID:27809470 PMCID:PMC5293171

 

SUSD2 expression in high-grade serous ovarian cancer correlates with increased patient survival and defective mesothelial clearance.

Sheets JN, Iwanicki M, Liu JF, Howitt BE, Hirsch MS, Gubbels JA, Drapkin R, Egland KA.
Oncogenesis. 2016; 5(10):e264.
PMID:27775699 PMCID:PMC5117850

 

Diet-induced obesity prolongs neuroinflammation and recruits CCR2(+) monocytes to the brain following herpes simplex virus (HSV)-1 latency in mice.

White KA, Hutton SR, Weimer JM, Sheridan PA.
Brain, behavior, and immunity. 2016; 57:68-78. NIHMSID: NIHMS796915
PMID:27311830 PMCID:PMC5287935

 

Midwest Science Festival: Exploring Students' and Parents' Participation in and Attitudes Toward Science

Dippel EA, Mechels KB, Griese ER, Laufmann RN, Weimer JM
South Dakota Medicine. 2016. 69(8):343-349
PMID:28806002

 

A novel mouse model for the identification of thioredoxin-1 protein interactions.

Booze ML, Hansen JM, Vitiello PF.
Free radical biology & medicine. 2016; 99:533-543. NIHMSID: NIHMS817985
PMID:27639450 PMCID:PMC5107173

 

Loss-of-Function Mutations in FRRS1L Lead to an Epileptic-Dyskinetic Encephalopathy

Madeo M, Stewart M, Sun Y, Sahir N, Wiethoff S, Chandrasekar I, Yarrow A, Rosenfeld JA, Yang Y, Cordeiro D, McCormick EM, Muraresku CC, Jepperson TN, McBeth LJ, Seidahmed MZ, El Khashab HY, Hamad M, Azzedine H, Clark K, Corrochano S, Wells S, Elting MW, Weiss MM, Burn S, Myers A, Landsverk M, Crotwell PL, Waisfisz Q, Wolf NI, Nolan PM, Padilla-Lopez S, Houlden H, Lifton R, Mane S, Singh BB, Falk MJ, Mercimek-Mahmutoglu S, Bilguvar K, Salih MA, Acevedo-Arozena A, Kruer MC
American Journal of Human Genetics.  2016. 2;98(6):1249-1255
PMID:27236917 PMCID:PMC4908178

 

Disruption of a cystine transporter downregulates expression of genes involved in sulfur regulation and cellular respiration.

Simpkins JA, Rickel KE, Madeo M, Ahlers BA, Carlisle GB, Nelson HJ, Cardillo AL, Weber EA, Vitiello PF, Pearce DA, Vitiello SP.
Biology open. 2016; 5(6):689-97.
PMID:27142334 PMCID:PMC4920189

 

Activation of GPR119 Stimulates Human β-Cell Replication and Neogenesis in Humanized Mice with Functional Human Islets

Ansarullah, Free C, Christopherson J, Chen Q, Gao J, Liu C, Naji A, Rabinovitch A, Guo Z
Diabetes Research. 2016. 2016;1620821
PMID:27413754 PMCID:PMC4927982

 

Metastatic model of HPV+ oropharyngeal squamous cell carcinoma demonstrates heterogeneity in tumor metastasis.

Vermeer DW, Coppock JD, Zeng E, Lee KM, Spanos WC, Onken MD, Uppaluri R, Lee JH, Vermeer PD.
Oncotarget. 2016; 7(17):24194-207.
PMID:27013584 PMCID:PMC5029694

 

An improved smaller biotin ligase for BioID proximity labeling.

Kim DI, Jensen SC, Noble KA, Kc B, Roux KH, Motamedchaboki K, Roux KJ.
Molecular biology of the cell. 2016; 27(8):1188-96.
PMID:26912792 PMCID:PMC4831873

 

Sonic Hedgehog promotes proliferation of Notch-dependent monociliated choroid plexus tumour cells.

Li L, Grausam KB, Wang J, Lun MP, Ohli J, Lidov HG, Calicchio ML, Zeng E, Salisbury JL, Wechsler-Reya RJ, Lehtinen MK, Schüller U, Zhao H.
Nature cell biology. 2016; 18(4):418-30. NIHMSID: NIHMS759136
PMID:26999738 PMCID:PMC4814324

 

Daily Enteral DHA Supplementation Alleviates Deficiency in Premature Infants.

Baack ML, Puumala SE, Messier SE, Pritchett DK, Harris WS.
Lipids. 2016; 51(4):423-33. NIHMSID: NIHMS757815
PMID:26846324 PMCID:PMC4818658

 

Maternal high-fat diet impairs cardiac function in offspring of diabetic pregnancy through metabolic stress and mitochondrial dysfunction.

Mdaki KS, Larsen TD, Wachal AL, Schimelpfenig MD, Weaver LJ, Dooyema SD, Louwagie EJ, Baack ML.
American journal of physiology. Heart and circulatory physiology. 2016; 310(6):H681-92.
PMID:26801311 PMCID:PMC4867345

 

Loss of Peripheral Protection in Pancreatic Islets by Proteolysis-Driven Impairment of VTCN1 (B7-H4) Presentation Is Associated with the Development of Autoimmune Diabetes.

Radichev IA, Maneva-Radicheva LV, Amatya C, Salehi M, Parker C, Ellefson J, Burn P, Savinov AY.
Journal of immunology (Baltimore, Md. : 1950). 2016; 196(4):1495-506. NIHMSID: NIHMS744363
PMID:26773144 PMCID:PMC4744569

 

Identification of Redox and Glucose-Dependent Txnip Protein Interactions.

Forred BJ, Neuharth S, Kim DI, Amolins MW, Motamedchaboki K, Roux KJ, Vitiello PF.
Oxidative medicine and cellular longevity. 2016; 2016:5829063.
PMID:27437069 PMCID:PMC4942636

 

Consequences of a Maternal High-Fat Diet and Late Gestation Diabetes on the Developing Rat Lung.

Baack ML, Forred BJ, Larsen TD, Jensen DN, Wachal AL, Khan MA, Vitiello PF.
PloS one. 2016; 11(8):e0160818.
PMID:27518105 PMCID:PMC4982689

 

2015

A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease.

Beraldi R, Chan CH, Rogers CS, Kovács AD, Meyerholz DK, Trantzas C, Lambertz AM, Darbro BW, Weber KL, White KA, Rheeden RV, Kruer MC, Dacken BA, Wang XJ, Davis BT, Rohret JA, Struzynski JT, Rohret FA, Weimer JM, Pearce DA.
Human molecular genetics. 2015; 24(22):6473-84.
PMID:26374845 PMCID:PMC4614707

 

MARCKS-dependent mucin clearance and lipid metabolism in ependymal cells are required for maintenance of forebrain homeostasis during aging.

Muthusamy N, Sommerville LJ, Moeser AJ, Stumpo DJ, Sannes P, Adler K, Blackshear PJ, Weimer JM, Ghashghaei HT.
Aging cell. 2015; 14(5):764-73.
PMID:26010231 PMCID:PMC4568964

 

CFAP54 is required for proper ciliary motility and assembly of the central pair apparatus in mice.

McKenzie CW, Craige B, Kroeger TV, Finn R, Wyatt TA, Sisson JH, Pavlik JA, Strittmatter L, Hendricks GM, Witman GB, Lee L.
Molecular biology of the cell. 2015; 26(18):3140-9.
PMID:26224312 PMCID:PMC4569307

 

What is the relationship between gestational age and docosahexaenoic acid (DHA) and arachidonic acid (ARA) levels?

Baack ML, Puumala SE, Messier SE, Pritchett DK, Harris WS.
Prostaglandins, leukotrienes, and essential fatty acids. 2015; 100:5-11. NIHMSID: NIHMS704991
PMID:26205427 PMCID:PMC4554773

 

The nucleus is an intracellular propagator of tensile forces in NIH 3T3 fibroblasts.

Alam SG, Lovett D, Kim DI, Roux KJ, Dickinson RB, Lele TP.
Journal of cell science. 2015; 128(10):1901-11.
PMID:25908852 PMCID:PMC4457156

 

Adam10 mediates the choice between principal cells and intercalated cells in the kidney.

Guo Q, Wang Y, Tripathi P, Manda KR, Mukherjee M, Chaklader M, Austin PF, Surendran K, Chen F.
Journal of the American Society of Nephrology : JASN. 2015; 26(1):149-59.
PMID:24904084 PMCID:PMC4279728

 

Beyond building better brains: bridging the docosahexaenoic acid (DHA) gap of prematurity.

Harris WS, Baack ML.
Journal of perinatology : official journal of the California Perinatal Association. 2015; 35(1):1-7. NIHMSID: NIHMS623925
PMID:25357095 PMCID:PMC4281288

 

Abbreviated exposure to hypoxia is sufficient to induce CNS dysmyelination, modulate spinal motor neuron composition, and impair motor development in neonatal mice.

Watzlawik JO, Kahoud RJ, O'Toole RJ, White KA, Ogden AR, Painter MM, Wootla B, Papke LM, Denic A, Weimer JM, Carey WA, Rodriguez M.
PloS one. 2015; 10(5):e0128007.
PMID:26020269 PMCID:PMC4447462

 

2014

Lysosome dysfunction in the pathogenesis of kidney diseases.

Surendran K, Vitiello SP, Pearce DA.
Pediatric nephrology (Berlin, Germany). 2014; 29(12):2253-61. NIHMSID: NIHMS539622
PMID:24217784 PMCID:PMC4018427

 

The human synaptic vesicle protein, SV2A, functions as a galactose transporter in Saccharomyces cerevisiae.

Madeo M, Kovács AD, Pearce DA.
The Journal of biological chemistry. 2014; 289(48):33066-71.
PMID:25326386 PMCID:PMC4246065

 

Thioredoxin-1 redox signaling regulates cell survival in response to hyperoxia.

Floen MJ, Forred BJ, Bloom EJ, Vitiello PF.
Free radical biology & medicine. 2014; 75:167-77. NIHMSID: NIHMS619506
PMID:25106706 PMCID:PMC4174305

 

Dichloroacetate enhances apoptotic cell death via oxidative damage and attenuates lactate production in metformin-treated breast cancer cells.

Haugrud AB, Zhuang Y, Coppock JD, Miskimins WK.
Breast cancer research and treatment. 2014; 147(3):539-50. NIHMSID: NIHMS627950
PMID:25212175 PMCID:PMC4184194

 

Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2.

Finn R, Evans CC, Lee L.
Neuroscience. 2014; 277:552-67. NIHMSID: NIHMS617242
PMID:25073043 PMCID:PMC4164581

 

Probing nuclear pore complex architecture with proximity-dependent biotinylation.

Kim DI, Birendra KC, Zhu W, Motamedchaboki K, Doye V, Roux KJ.
Proceedings of the National Academy of Sciences of the United States of America. 2014; 111(24):E2453-61.
PMID:24927568 PMCID:PMC4066523

 

2013

Large animal models for Batten disease: a review.

Weber K, Pearce DA.
Journal of child neurology. 2013; 28(9):1123-7. NIHMSID: NIHMS569666
PMID:24014507 PMCID:PMC4009683

 

Treatment of the Ppt1(-/-) mouse model of infantile neuronal ceroid lipofuscinosis with the N-methyl-D-aspartate (NMDA) receptor antagonist memantine.

Finn R, Kovács AD, Pearce DA.
Journal of child neurology. 2013; 28(9):1159-68. NIHMSID: NIHMS569670
PMID:24014511 PMCID:PMC4017336

 

Seizure susceptibility, phenotype, and resultant growth delay in the nclf and mnd mouse models of neuronal ceroid lipofuscinoses.

Kriscenski-Perry E, Kovács AD, Pearce DA.
Journal of child neurology. 2013; 28(9):1137-41. NIHMSID: NIHMS569665
PMID:23838029 PMCID:PMC4078599