Username

Password

Forgot Password?

Preview

Sign-in free and Explore the Exciting World of BiomedExperts:

Over 1.500.000 Profiles
More than 1.800 Organizations worldwide
State of the Art Network Visualizations

Manage your own Profile
Locate Experts in your Country/Region
Locate Experts in your 1. and 2. Level Network
Connect to Experts Worldwide

NetworkView

Nina Raben

This is a preview profile on BiomedExperts - the first literature-based scientific social network. It brings the right researchers together and allows them to collaborate online. Collexis and Dell provide the BiomedExperts network of +1.5 Million pre-calculated profiles free of charge to researchers worldwide.

Research Profile (preview)

Disorders

Chemicals & Drugs

Living Beings

Physiology

Concepts & Ideas

Molecular Sequence Data

Anatomy

Sign-in to see full Profile

Network (preview)

Co-Publications
Name
49
Plotz, Paul
14
Nagaraju, Kanneboyina
6
Mattaliano, Robert
5
Ralston, Evelyn
5
Fukuda, Tokiko
4
Dwivedi, Sunita
4
Lu, Nina
4
Roberts, Ashley
4
Thurberg, Beth
4
Yan, Bo
4
Zaal, Kristien
3
Miller, Frederick
3
Rom, William
3
Rosen, Antony
3
Takikita, Shoichi

Sign-in to see all Coauthors

Publications

TOP 3
Takikita Shoichi; Myerowitz Rachel; Schreiner Cynthia; Baum Rebecca; Raben Nina; Plotz Paul H
The values and limits of an in vitro model of Pompe disease: the best laid schemes o' mice an' men...
Takikita Shoichi; Myerowitz Rachel; Zaal Kristien; Raben Nina; Plotz Paul H
Murine muscle cell models for Pompe disease and their use in studying therapeutic approaches.
Raben Nina; Shea Lauren; Hill Victoria; Plotz Paul
Monitoring autophagy in lysosomal storage disorders.

Sign-in to see all Publications

Sign in free and see...

Visualized networks:

See your personal network in
sophisticated graphical views

GeoTargeted Searches:

Locate experts around the world
and connect with global collaborators

Research Profiles:

See the visualized research activity
of experts around the globe

Sign-in to see more

Geonetwork of Nina Raben (preview)

Cities where this author has publications
Cities where co-authors of this author have publications

Flash Player Required

Sign-in to see more

All Publications

2009: Takikita Shoichi; Myerowitz Rachel; Schreiner Cynthia; Baum Rebecca; Raben Nina; Plotz Paul H
The values and limits of an in vitro model of Pompe disease: the best laid schemes o' mice an' men...
Autophagy 2009;5(5):729-31.
2009: Takikita Shoichi; Myerowitz Rachel; Zaal Kristien; Raben Nina; Plotz Paul H
Murine muscle cell models for Pompe disease and their use in studying therapeutic approaches.
Molecular genetics and metabolism 2009;96(4):208-17.
2009: Raben Nina; Shea Lauren; Hill Victoria; Plotz Paul
Monitoring autophagy in lysosomal storage disorders.
Methods in enzymology 2009;453():417-49.
2008: Ralston E; Swaim B; Czapiga M; Hwu W-L; Chien Y-H; Pittis M G; Bembi B; Schwartz O; Plotz P; Raben N
Detection and imaging of non-contractile inclusions and sarcomeric anomalies in skeletal muscle by second harmonic generation combined with two-photon excited fluorescence.
Journal of structural biology 2008;162(3):500-8.
2007: Raben Nina; Takikita Shoichi; Pittis Maria G; Bembi Bruno; Marie Suely K N; Roberts Ashley; Page Laura; Kishnani Priya S; Schoser Benedikt G H; Chien Yin-Hsiu; Ralston Evelyn; Nagaraju Kanneboyina; Plotz Paul H
Deconstructing Pompe disease by analyzing single muscle fibers: to see a world in a grain of sand...
Autophagy 2007;3(6):546-52.
2007: Levine Stuart M; Raben Nina; Xie Dan; Askin Frederic B; Tuder Rubin; Mullins Marissa; Rosen Antony; Casciola-Rosen Livia A
Novel conformation of histidyl-transfer RNA synthetase in the lung: the target tissue in Jo-1 autoantibody-associated myositis.
Arthritis and rheumatism 2007;56(8):2729-39.
2007: Raben N; Roberts A; Plotz P H
Role of autophagy in the pathogenesis of Pompe disease.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology / edited by the Gaetano Conte Academy for the study of striated muscle diseases 2007;26(1):45-8.
2007: Fukuda Tokiko; Roberts Ashley; Plotz Paul H; Raben Nina
Acid alpha-glucosidase deficiency (Pompe disease).
Current neurology and neuroscience reports 2007;7(1):71-7.
2006: Fukuda Tokiko; Ahearn Meghan; Roberts Ashley; Mattaliano Robert J; Zaal Kristien; Ralston Evelyn; Plotz Paul H; Raben Nina
Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease.
Molecular therapy : the journal of the American Society of Gene Therapy 2006;14(6):831-9.
2006: Wang Wei; Parker Gretchen E; Skurat Alexander V; Raben Nina; DePaoli-Roach Anna A; Roach Peter J
Relationship between glycogen accumulation and the laforin dual specificity phosphatase.
Biochemical and biophysical research communications 2006;350(3):588-92.
2006: Fukuda Tokiko; Roberts Ashley; Ahearn Meghan; Zaal Kristien; Ralston Evelyn; Plotz Paul H; Raben Nina
Autophagy and lysosomes in Pompe disease.
Autophagy 2006;2(4):318-20.
2006: Fukuda Tokiko; Ewan Lindsay; Bauer Martina; Mattaliano Robert J; Zaal Kristien; Ralston Evelyn; Plotz Paul H; Raben Nina
Dysfunction of endocytic and autophagic pathways in a lysosomal storage disease.
Annals of neurology 2006;59(4):700-8.
2005: Zhu Yunxiang; Li Xuemei; McVie-Wylie Alison; Jiang Canwen; Thurberg Beth L; Raben Nina; Mattaliano Robert J; Cheng Seng H
Carbohydrate-remodelled acid alpha-glucosidase with higher affinity for the cation-independent mannose 6-phosphate receptor demonstrates improved delivery to muscles of Pompe mice.
The Biochemical journal 2005;389(Pt 3):619-28.
2005: Nagaraju Kanneboyina; Casciola-Rosen Livia; Lundberg Ingrid; Rawat Rashmi; Cutting Shawna; Thapliyal Rachana; Chang Jason; Dwivedi Sunita; Mitsak Megan; Chen Yi-Wen; Plotz Paul; Rosen Antony; Hoffman Eric; Raben Nina
Activation of the endoplasmic reticulum stress response in autoimmune myositis: potential role in muscle fiber damage and dysfunction.
Arthritis and rheumatism 2005;52(6):1824-35.
2005: Raben Nina; Fukuda Tokiko; Gilbert Abigail L; de Jong Deborah; Thurberg Beth L; Mattaliano Robert J; Meikle Peter; Hopwood John J; Nagashima Kunio; Nagaraju Kanneboyina; Plotz Paul H
Replacing acid alpha-glucosidase in Pompe disease: recombinant and transgenic enzymes are equipotent, but neither completely clears glycogen from type II muscle fibers.
Molecular therapy : the journal of the American Society of Gene Therapy 2005;11(1):48-56.
2004: Zhu Yunxiang; Li Xuemei; Kyazike Josephine; Zhou Qun; Thurberg Beth L; Raben Nina; Mattaliano Robert J; Cheng Seng H
Conjugation of mannose 6-phosphate-containing oligosaccharides to acid alpha-glucosidase improves the clearance of glycogen in pompe mice.
The Journal of biological chemistry 2004;279(48):50336-41.
2003: Raben N; Danon M; Gilbert A L; Dwivedi S; Collins B; Thurberg B L; Mattaliano R J; Nagaraju K; Plotz P H
Enzyme replacement therapy in the mouse model of Pompe disease.
Molecular genetics and metabolism 2003;80(1-2):159-69.
2003: Raben Nina; Nagaraju Kanneboyina; Lee Alicia; Lu Nina; Rivera Yesenia; Jatkar Tejas; Hopwood John J; Plotz Paul H
Induction of tolerance to a recombinant human enzyme, acid alpha-glucosidase, in enzyme deficient knockout mice.
Transgenic research 2003;12(2):171-8.
2003: Chae Jae Jin; Komarow Hirsh D; Cheng Jun; Wood Geryl; Raben Nina; Liu P Paul; Kastner Daniel L
Targeted disruption of pyrin, the FMF protein, causes heightened sensitivity to endotoxin and a defect in macrophage apoptosis.
Molecular cell 2003;11(3):591-604.
2002: Raben Nina; Jatkar Tejas; Lee Alicia; Lu Nina; Dwivedi Sunita; Nagaraju Kanneboyina; Plotz Paul H
Glycogen stored in skeletal but not in cardiac muscle in acid alpha-glucosidase mutant (Pompe) mice is highly resistant to transgene-encoded human enzyme.
Molecular therapy : the journal of the American Society of Gene Therapy 2002;6(5):601-8.
2002: Martiniuk Frank; Chen Agnes; Mack Adra; Donnabella Vincent; Slonim Alfred; Bulone Linda; Arvanitopoulos Eleni; Raben Nina; Plotz Paul; Rom William N
Helios gene gun particle delivery for therapy of acid maltase deficiency.
DNA and cell biology 2002;21(10):717-25.
2002: Howard O M Zack; Dong Hui Fang; Yang De; Raben Nina; Nagaraju Kanneboyina; Rosen Antony; Casciola-Rosen Livia; Härtlein Michael; Kron Michael; Yang David; Yiadom Kwabena; Dwivedi Sunita; Plotz Paul H; Oppenheim Joost J
Histidyl-tRNA synthetase and asparaginyl-tRNA synthetase, autoantigens in myositis, activate chemokine receptors on T lymphocytes and immature dendritic cells.
The Journal of experimental medicine 2002;196(6):781-91.
2002: Yan Bo; Raben Nina; Plotz Paul
The human acid alpha-glucosidase gene is a novel target of the Notch-1/Hes-1 signaling pathway.
The Journal of biological chemistry 2002;277(33):29760-4.
2002: Fraites Thomas J; Schleissing Mary R; Shanely R Andrew; Walter Glenn A; Cloutier Denise A; Zolotukhin Irene; Pauly Daniel F; Raben Nina; Plotz Paul H; Powers Scott K; Kessler Paul D; Byrne Barry J
Correction of the enzymatic and functional deficits in a model of Pompe disease using adeno-associated virus vectors.
Molecular therapy : the journal of the American Society of Gene Therapy 2002;5(5 Pt 1):571-8.
2002: Raben Nina; Plotz Paul; Byrne Barry J
Acid alpha-glucosidase deficiency (glycogenosis type II, Pompe disease).
Current molecular medicine 2002;2(2):145-66.
2002: Yan Bo; Raben Nina; Plotz Paul H
Hes-1, a known transcriptional repressor, acts as a transcriptional activator for the human acid alpha-glucosidase gene in human fibroblast cells.
Biochemical and biophysical research communications 2002;291(3):582-7.
2001: Raben N; Lu N; Nagaraju K; Rivera Y; Lee A; Yan B; Byrne B; Meikle P J; Umapathysivam K; Hopwood J J; Plotz P H
Conditional tissue-specific expression of the acid alpha-glucosidase (GAA) gene in the GAA knockout mice: implications for therapy.
Human molecular genetics 2001;10(19):2039-47.
2001: Yan B; Raben N; Lu N; Plotz P H
Identification and characterization of a tissue-specific silencer element in the first intron of the human acid maltase gene.
Human genetics 2001;109(2):186-90.
2001: Raben N; Danon M; Lu N; Lee E; Shliselfeld L; Skurat A V; Roach P J; Lawrence J C; Musumeci O; Shanske S; DiMauro S; Plotz P
Surprises of genetic engineering: a possible model of polyglucosan body disease.
Neurology 2001;56(12):1739-45.
2001: Pauly D F; Fraites T J; Toma C; Bayes H S; Huie M L; Hirschhorn R; Plotz P H; Raben N; Kessler P D; Byrne B J
Intercellular transfer of the virally derived precursor form of acid alpha-glucosidase corrects the enzyme deficiency in inherited cardioskeletal myopathy Pompe disease.
Human gene therapy 2001;12(5):527-38.
2001: Yan B; Heus J; Lu N; Nichols R C; Raben N; Plotz P H
Transcriptional regulation of the human acid alpha-glucosidase gene. Identification of a repressor element and its transcription factors Hes-1 and YY1.
The Journal of biological chemistry 2001;276(3):1789-93.
2000: Martiniuk F; Chen A; Donnabella V; Arvanitopoulos E; Slonim A E; Raben N; Plotz P; Rom W N
Correction of glycogen storage disease type II by enzyme replacement with a recombinant human acid maltase produced by over-expression in a CHO-DHFR(neg) cell line.
Biochemical and biophysical research communications 2000;276(3):917-23.
2000: Nagaraju K; Raben N; Loeffler L; Parker T; Rochon P J; Lee E; Danning C; Wada R; Thompson C; Bahtiyar G; Craft J; Hooft Van Huijsduijnen R; Plotz P
Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specific autoantibodies.
Proceedings of the National Academy of Sciences of the United States of America 2000;97(16):9209-14.
2000: Raben N; Nagaraju K; Lee E; Plotz P
Modulation of disease severity in mice with targeted disruption of the acid alpha-glucosidase gene.
Neuromuscular disorders : NMD 2000;10(4-5):283-91.
1999: Nagaraju K; Raben N; Villalba M L; Danning C; Loeffler L A; Lee E; Tresser N; Abati A; Fetsch P; Plotz P H
Costimulatory markers in muscle of patients with idiopathic inflammatory myopathies and in cultured muscle cells.
Clinical immunology (Orlando, Fla.) 1999;92(2):161-9.
1999: Amalfitano A; McVie-Wylie A J; Hu H; Dawson T L; Raben N; Plotz P; Chen Y T
Systemic correction of the muscle disorder glycogen storage disease type II after hepatic targeting of a modified adenovirus vector encoding human acid-alpha-glucosidase.
Proceedings of the National Academy of Sciences of the United States of America 1999;96(16):8861-6.
1999: Silver K; Walston J; Yang Y; Pratley R; Ravussin E; Raben N; Shuldiner A R
Molecular scanning of the beta-3-adrenergic receptor gene in Pima Indians and Caucasians.
Diabetes/metabolism research and reviews 1999;15(3):175-80.
1999: Raben N; Lee E; Lee L; Hirschhorn R; Plotz P H
Novel mutations in African American patients with glycogen storage disease Type II. Mutations in brief no. 209. Online.
Human mutation 1999;13(1):83-4.
1998: Nagaraju K; Raben N; Merritt G; Loeffler L; Kirk K; Plotz P
A variety of cytokines and immunologically relevant surface molecules are expressed by normal human skeletal muscle cells under proinflammatory stimuli.
Clinical and experimental immunology 1998;113(3):407-14.
1998: Martiniuk F; Chen A; Mack A; Arvanitopoulos E; Chen Y; Rom W N; Codd W J; Hanna B; Alcabes P; Raben N; Plotz P
Carrier frequency for glycogen storage disease type II in New York and estimates of affected individuals born with the disease.
American journal of medical genetics 1998;79(1):69-72.
1998: Raben N; Nagaraju K; Lee E; Kessler P; Byrne B; Lee L; LaMarca M; King C; Ward J; Sauer B; Plotz P
Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II.
The Journal of biological chemistry 1998;273(30):19086-92.
1998: Becker J A; Vlach J; Raben N; Nagaraju K; Adams E M; Hermans M M; Reuser A J; Brooks S S; Tifft C J; Hirschhorn R; Huie M L; Nicolino M; Plotz P H
The African origin of the common mutation in African American patients with glycogen-storage disease type II.
American journal of human genetics 1998;62(4):991-4.
1997: Adams E M; Becker J A; Griffith L; Segal A; Plotz P H; Raben N
Glycogenosis type II: a juvenile-specific mutation with an unusual splicing pattern and a shared mutation in African Americans.
Human mutation 1997;10(2):128-34.
1996: Pras E; Sood R; Raben N; Aksentijevich I; Chen X; Kastner D L
Genomic organization of SLC3A1, a transporter gene mutated in cystinuria.
Genomics 1996;36(1):163-7.
1996: Raben N; Nichols R C; Martiniuk F; Plotz P H
A model of mRNA splicing in adult lysosomal storage disease (glycogenosis type II).
Human molecular genetics 1996;5(7):995-1000.
1996: Nichols R C; Rudolphi O; Ek B; Exelbert R; Plotz P H; Raben N
Glycogenosis type VII (Tarui disease) in a Swedish family: two novel mutations in muscle phosphofructokinase gene (PFK-M) resulting in intron retentions.
American journal of human genetics 1996;59(1):59-65.
1995: Pras E; Raben N; Golomb E; Arber N; Aksentijevich I; Schapiro J M; Harel D; Katz G; Liberman U; Pras M
Mutations in the SLC3A1 transporter gene in cystinuria.
American journal of human genetics 1995;56(6):1297-303.
1995: O'Hanlon T P; Raben N; Miller F W
A novel gene oriented in a head-to-head configuration with the human histidyl-tRNA synthetase (HRS) gene encodes an mRNA that predicts a polypeptide homologous to HRS.
Biochemical and biophysical research communications 1995;210(2):556-66.
1995: Plotz P H; Rider L G; Targoff I N; Raben N; O'Hanlon T P; Miller F W
NIH conference. Myositis: immunologic contributions to understanding cause, pathogenesis, and therapy.
Annals of internal medicine 1995;122(9):715-24.
1995: Nichols R C; Raben N; Boerkoel C F; Plotz P H
Human isoleucyl-tRNA synthetase: sequence of the cDNA, alternative mRNA splicing, and the characteristics of an unusually long C-terminal extension.
Gene 1995;155(2):299-304.
1995: Boerkoel C F; Exelbert R; Nicastri C; Nichols R C; Miller F W; Plotz P H; Raben N
Leaky splicing mutation in the acid maltase gene is associated with delayed onset of glycogenosis type II.
American journal of human genetics 1995;56(4):887-97.
1995: Raben N; Exelbert R; Spiegel R; Sherman J B; Nakajima H; Plotz P; Heinisch J
Functional expression of human mutant phosphofructokinase in yeast: genetic defects in French Canadian and Swiss patients with phosphofructokinase deficiency.
American journal of human genetics 1995;56(1):131-41.
1995: Raben N; Nichols R C; Boerkoel C; Plotz P
Genetic defects in patients with glycogenosis type II (acid maltase deficiency).
Muscle & nerve 1995;3():S70-4.
1995: Raben N; Sherman J B; Adams E; Nakajima H; Argov Z; Plotz P
Various classes of mutations in patients with phosphofructokinase deficiency (Tarui's disease).
Muscle & nerve 1995;3():S35-8.
1995: Raben N; Sherman J B
Mutations in muscle phosphofructokinase gene.
Human mutation 1995;6(1):1-6.
1994: Nichols R C; Raben N
Hints for direct sequencing of PCR-generated single-stranded DNA.
BioTechniques 1994;17(3):412, 414.
1994: Raben N; Nichols R; Dohlman J; McPhie P; Sridhar V; Hyde C; Leff R; Plotz P
A motif in human histidyl-tRNA synthetase which is shared among several aminoacyl-tRNA synthetases is a coiled-coil that is essential for enzymatic activity and contains the major autoantigenic epitope.
The Journal of biological chemistry 1994;269(39):24277-83.
1994: Sherman J B; Raben N; Nicastri C; Argov Z; Nakajima H; Adams E M; Eng C M; Cowan T M; Plotz P H
Common mutations in the phosphofructokinase-M gene in Ashkenazi Jewish patients with glycogenesis VII--and their population frequency.
American journal of human genetics 1994;55(2):305-13.
1994: Argov Z; Barash V; Soffer D; Sherman J; Raben N
Late-onset muscular weakness in phosphofructokinase deficiency due to exon 5/intron 5 junction point mutation: a unique disorder or the natural course of this glycolytic disorder?
Neurology 1994;44(6):1097-100.
1994: Rider L G; Raben N; Miller L; Jelsema C
The cDNAs encoding two forms of the LYN protein tyrosine kinase are expressed in rat mast cells and human myeloid cells.
Gene 1994;138(1-2):219-22.
1993: Raben N; Sherman J; Miller F; Mena H; Plotz P
A 5' splice junction mutation leading to exon deletion in an Ashkenazic Jewish family with phosphofructokinase deficiency (Tarui disease).
The Journal of biological chemistry 1993;268(7):4963-7.
1992: Barbetti F; Gejman P V; Taylor S I; Raben N; Cama A; Bonora E; Pizzo P; Moghetti P; Muggeo M; Roth J
Detection of mutations in insulin receptor gene by denaturing gradient gel electrophoresis.
Diabetes 1992;41(4):408-15.
1992: Raben N; Borriello F; Amin J; Horwitz R; Fraser D; Plotz P
Human histidyl-tRNA synthetase: recognition of amino acid signature regions in class 2a aminoacyl-tRNA synthetases.
Nucleic acids research 1992;20(5):1075-81.
1991: Raben N; Barbetti F; Cama A; Lesniak M A; Lillioja S; Zimmet P; Serjeantson S W; Taylor S I; Roth J
Normal coding sequence of insulin gene in Pima Indians and Nauruans, two groups with highest prevalence of type II diabetes.
Diabetes 1991;40(1):118-22.
1990: Barbetti F; Raben N; Kadowaki T; Cama A; Accili D; Gabbay K H; Merenich J A; Taylor S I; Roth J
Two unrelated patients with familial hyperproinsulinemia due to a mutation substituting histidine for arginine at position 65 in the proinsulin molecule: identification of the mutation by direct sequencing of genomic deoxyribonucleic acid amplified by polymerase chain reaction.
The Journal of clinical endocrinology and metabolism 1990;71(1):164-9.

Sign-in to see more