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Kirby Smith
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20
Watkins, Paul
14
Moser, Ann
10
Lu, Jyh-Feng
10
Dover, George
9
Moser, Hugo
9
Scott, Alan
9
McGuinness, Martina
8
Heinzer, Ann
7
Braiterman, Lelita
6
Berkovitz, Gary
6
Migeon, Claude
6
Kemp, Stephan
6
Steinberg, Steven
6
Powers, James
5
Pei, Zhengtong
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All Publications
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2009: Duggan P S; Siegel A W; Blass D M; Bok H; Coyle J T; Faden R; Finkel J; Gearhart J D; Greely H T; Hillis A; Hoke A; Johnson R; Johnston M; Kahn J; Kerr D; King P; Kurtzberg J; Liao S M; McDonald J W; McKhann G; Nelson K B; Rao M; Regenberg A; Smith K; Solter D; Song H; Sugarman J; Traystman R J; Vescovi A; Yanofski J; Young W; Mathews D J H
Unintended changes in cognition, mood, and behavior arising from cell-based interventions for neurological conditions: ethical challenges.
The American journal of bioethics : AJOB 2009;9(5):31-6.
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2009: Regenberg Alan; Mathews Debra J H; Blass David M; Bok Hilary; Coyle Joseph T; Duggan Patrick; Faden Ruth; Finkel Julia; Gearhart John D; Hillis Argye; Hoke Ahmet; Johnson Richard; Johnston Michael; Kahn Jeffrey; Kerr Douglas; King Patricia; Kurtzberg Joanne; Liao S Matthew; McDonald John W; McKhann Guy; Nelson Karin B; Rao Mahendra; Siegel Andrew W; Smith Kirby; Solter Davor; Song Hongjun; Sugarman Jeremy; Vescovi Angelo; Young Wise; Greely Henry T; Traystman Richard J
The role of animal models in evaluating reasonable safety and efficacy for human trials of cell-based interventions for neurologic conditions.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism 2009;29(1):1-9.
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2008: Mathews D J H; Sugarman J; Bok H; Blass D M; Coyle J T; Duggan P; Finkel J; Greely H T; Hillis A; Hoke A; Johnson R; Johnston M; Kahn J; Kerr D; Kurtzberg J; Liao S M; McDonald J W; McKhann G; Nelson K B; Rao M; Regenberg A; Siegel A W; Smith K; Solter D; Song H; Vescovi A; Young W; Gearhart J D; Faden R
Cell-based interventions for neurologic conditions: ethical challenges for early human trials.
Neurology 2008;71(4):288-93.
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2007: Lu Jyh-Feng; Barron-Casella Emily; Deering Rebecca; Heinzer Ann K; Moser Ann B; deMesy Bentley Karen L; Wand Gary S; C McGuinness Martina; Pei Zhengtong; Watkins Paul A; Pujol Aurora; Smith Kirby D; Powers James M
The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage.
Laboratory investigation; a journal of technical methods and pathology 2007;87(3):261-72.
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2006: Keefer Jeffrey R; Schneidereith Tonya A; Mays Abbie; Purvis Shirley H; Dover George J; Smith Kirby D
Role of cyclic nucleotides in fetal hemoglobin induction in cultured CD34+ cells.
Experimental hematology 2006;34(9):1151-61.
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2005: Powers James M; Pei Zhengtong; Heinzer Ann K; Deering Rebecca; Moser Ann B; Moser Hugo W; Watkins Paul A; Smith Kirby D
Adreno-leukodystrophy: oxidative stress of mice and men.
Journal of neuropathology and experimental neurology 2005;64(12):1067-79.
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2004: Jia Zhenzhen; Pei Zhengtong; Li Yuanyuan; Wei Liumei; Smith Kirby D; Watkins Paul A
X-linked adrenoleukodystrophy: role of very long-chain acyl-CoA synthetases.
Molecular genetics and metabolism 2004;83(1-2):117-27.
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2003: Pei Zhengtong; Oey Nadia A; Zuidervaart Maartje M; Jia Zhenzhen; Li Yuanyuan; Steinberg Steven J; Smith Kirby D; Watkins Paul A
The acyl-CoA synthetase "bubblegum" (lipidosin): further characterization and role in neuronal fatty acid beta-oxidation..
The Journal of biological chemistry 2003;278(47):47070-8.
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2003: Heinzer Ann K; Watkins Paul A; Lu Jyh-Feng; Kemp Stephan; Moser Ann B; Li Yuan Yuan; Mihalik Stephanie; Powers James M; Smith Kirby D
A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy.
Human molecular genetics 2003;12(10):1145-54.
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2003: McGuinness M C; Lu J-F; Zhang H-P; Dong G-X; Heinzer A K; Watkins P A; Powers J; Smith K D
Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy.
Molecular and cellular biology 2003;23(2):744-53.
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2003: Heinzer Ann K; McGuinness Martina C; Lu Jyh-Feng; Stine O Colin; Wei Heming; Van der Vlies Mark; Dong Gao-Xiang; Powers James; Watkins Paul A; Smith Kirby D
Mouse models and genetic modifiers in X-linked adrenoleukodystrophy.
Advances in experimental medicine and biology 2003;544():75-93.
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2002: Li Xiaoling; Baumgart Eveline; Dong Gao-Xiang; Morrell James C; Jimenez-Sanchez Gerardo; Valle David; Smith Kirby D; Gould Stephen J
PEX11alpha is required for peroxisome proliferation in response to 4-phenylbutyrate but is dispensable for peroxisome proliferator-activated receptor alpha-mediated peroxisome proliferation.
Molecular and cellular biology 2002;22(23):8226-40.
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2002: Heinzer Ann K; Kemp Stephan; Lu Jyh-Feng; Watkins Paul A; Smith Kirby D
Mouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophy.
The Journal of biological chemistry 2002;277(32):28765-73.
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2002: Mihalik Stephanie J; Steinberg Steven J; Pei Zhengtong; Park Joseph; Kim Do G; Heinzer Ann K; Dacremont Georges; Wanders Ronald J A; Cuebas Dean A; Smith Kirby D; Watkins Paul A
Participation of two members of the very long-chain acyl-CoA synthetase family in bile acid synthesis and recycling.
The Journal of biological chemistry 2002;277(27):24771-9.
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2002: Corzo Deyanira; Gibson William; Johnson Kisha; Mitchell Grant; LePage Guy; Cox Gerald F; Casey Robin; Zeiss Carolyn; Tyson Heidi; Cutting Garry R; Raymond Gerald V; Smith Kirby D; Watkins Paul A; Moser Ann B; Moser Hugo W; Steinberg Steven J
Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: a novel neonatal phenotype similar to peroxisomal biogenesis disorders.
American journal of human genetics 2002;70(6):1520-31.
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2001: Ito M; Blumberg B M; Mock D J; Goodman A D; Moser A B; Moser H W; Smith K D; Powers J M
Potential environmental and host participants in the early white matter lesion of adreno-leukodystrophy: morphologic evidence for CD8 cytotoxic T cells, cytolysis of oligodendrocytes, and CD1-mediated lipid antigen presentation.
Journal of neuropathology and experimental neurology 2001;60(10):1004-19.
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2001: McGuinness M C; Zhang H P; Smith K D
Evaluation of pharmacological induction of fatty acid beta-oxidation in X-linked adrenoleukodystrophy.
Molecular genetics and metabolism 2001;74(1-2):256-63.
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2001: Chang Y P; Littera R; Garau R; Smith K D; Dover G J; Iannelli S; Cacace E; Contu L
The role of heterocellular hereditary persistence of fetal haemoglobin in beta(0)-thalassaemia intermedia.
British journal of haematology 2001;114(4):899-906.
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2001: Bezman L; Moser A B; Raymond G V; Rinaldo P; Watkins P A; Smith K D; Kass N E; Moser H W
Adrenoleukodystrophy: incidence, new mutation rate, and results of extended family screening.
Annals of neurology 2001;49(4):512-7.
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2000: Steinberg S J; Morgenthaler J; Heinzer A K; Smith K D; Watkins P A
Very long-chain acyl-CoA synthetases. Human "bubblegum" represents a new family of proteins capable of activating very long-chain fatty acids.
The Journal of biological chemistry 2000;275(45):35162-9.
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2000: McGuinness M C; Wei H; Smith K D
Therapeutic developments in peroxisome biogenesis disorders.
Expert opinion on investigational drugs 2000;9(9):1985-92.
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2000: Lachtermacher M B; Seuanez H N; Moser H W; Smith K D
One-step multiplex PCR strategy for identification of mutations by SSCP and DNA sequencing.
BioTechniques 2000;29(2):234-6.
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2000: Wei H; Kemp S; McGuinness M C; Moser A B; Smith K D
Pharmacological induction of peroxisomes in peroxisome biogenesis disorders.
Annals of neurology 2000;47(3):286-96.
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2000: Watkins P A; Lu J F; Braiterman L T; Steinberg S J; Smith K D
Disruption of a yeast very-long-chain acyl-CoA synthetase gene simulates the cellular phenotype of X-linked adrenoleukodystrophy.
Cell biochemistry and biophysics 2000;32 Spring():333-7.
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2000: Lachtermacher M B; Seuánez H N; Moser A B; Moser H W; Smith K D
Determination of 30 X-linked adrenoleukodystrophy mutations, including 15 not previously described.
Human mutation 2000;15(4):348-53.
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1999: Smith K D; Kemp S; Braiterman L T; Lu J F; Wei H M; Geraghty M; Stetten G; Bergin J S; Pevsner J; Watkins P A
X-linked adrenoleukodystrophy: genes, mutations, and phenotypes.
Neurochemical research 1999;24(4):521-35.
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1999: Moser H W; Kemp S; Smith K D
Mutational analysis and the pathogenesis of variant X-linked adrenoleukodystrophy phenotypes.
Archives of neurology 1999;56(3):273-5.
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1999: Braiterman L T; Watkins P A; Moser A B; Smith K D
Peroxisomal very long chain fatty acid beta-oxidation activity is determined by the level of adrenodeukodystrophy protein (ALDP) expression.
Molecular genetics and metabolism 1999;66(2):91-9.
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1999: Bergen A W; Wang C Y; Tsai J; Jefferson K; Dey C; Smith K D; Park S C; Tsai S J; Goldman D
An Asian-Native American paternal lineage identified by RPS4Y resequencing and by microsatellite haplotyping.
Annals of human genetics 1999;63(Pt 1):63-80.
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1999: McGuinness M C; Smith K D
Cerebral inflammation in X-linked adrenoleukodystrophy.
Archivum immunologiae et therapiae experimentalis 1999;47(5):281-7.
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1998: Kemp S; Wei H M; Lu J F; Braiterman L T; McGuinness M C; Moser A B; Watkins P A; Smith K D
Gene redundancy and pharmacological gene therapy: implications for X-linked adrenoleukodystrophy.
Nature medicine 1998;4(11):1261-8.
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1998: Watkins P A; Lu J F; Steinberg S J; Gould S J; Smith K D; Braiterman L T
Disruption of the Saccharomyces cerevisiae FAT1 gene decreases very long-chain fatty acyl-CoA synthetase activity and elevates intracellular very long-chain fatty acid concentrations.
The Journal of biological chemistry 1998;273(29):18210-9.
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1998: Braiterman L T; Zheng S; Watkins P A; Geraghty M T; Johnson G; McGuinness M C; Moser A B; Smith K D
Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins.
Human molecular genetics 1998;7(2):239-47.
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1997: Lu J F; Lawler A M; Watkins P A; Powers J M; Moser A B; Moser H W; Smith K D
A mouse model for X-linked adrenoleukodystrophy.
Proceedings of the National Academy of Sciences of the United States of America 1997;94(17):9366-71.
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1997: McGuinness M C; Powers J M; Bias W B; Schmeckpeper B J; Segal A H; Gowda V C; Wesselingh S L; Berger J; Griffin D E; Smith K D
Human leukocyte antigens and cytokine expression in cerebral inflammatory demyelinative lesions of X-linked adrenoleukodystrophy and multiple sclerosis.
Journal of neuroimmunology 1997;75(1-2):174-82.
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1997: Chang Y P; Maier-Redelsperger M; Smith K D; Contu L; Ducroco R; de Montalembert M; Belloy M; Elion J; Dover G J; Girot R
The relative importance of the X-linked FCP locus and beta-globin haplotypes in determining haemoglobin F levels: a study of SS patients homozygous for beta S haplotypes.
British journal of haematology 1997;96(4):806-14.
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1995: McGuinness M C; Griffin D E; Raymond G V; Washington C A; Moser H W; Smith K D
Tumor necrosis factor-alpha and X-linked adrenoleukodystrophy.
Journal of neuroimmunology 1995;61(2):161-9.
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1995: Watkins P A; Gould S J; Smith M A; Braiterman L T; Wei H M; Kok F; Moser A B; Moser H W; Smith K D
Altered expression of ALDP in X-linked adrenoleukodystrophy.
American journal of human genetics 1995;57(2):292-301.
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1995: Moser H W; Powers J M; Smith K D
Adrenoleukodystrophy: molecular genetics, pathology, and Lorenzo's oil.
Brain pathology (Zurich, Switzerland) 1995;5(3):259-66.
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1995: Chang Y C; Smith K D; Moore R D; Serjeant G R; Dover G J
An analysis of fetal hemoglobin variation in sickle cell disease: the relative contributions of the X-linked factor, beta-globin haplotypes, alpha-globin gene number, gender, and age.
Blood 1995;85(4):1111-7.
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1994: Chang Y P; Smith K D; Dover G J
Dinucleotide repeat polymorphisms at the DXS85, DXS16 and DXS43 loci.
Human molecular genetics 1994;3(6):1029.
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1994: Fechner P Y; Rosenberg C; Stetten G; Cargile C B; Pearson P L; Smith K D; Migeon C J; Berkovitz G D
Nonrandom inactivation of the Y-bearing X chromosome in a 46,XX individual: evidence for the etiology of 46,XX true hermaphroditism.
Cytogenetics and cell genetics 1994;66(1):22-6.
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1993: Fechner P Y; Marcantonio S M; Ogata T; Rosales T O; Smith K D; Goodfellow P N; Migeon C J; Berkovitz G D
Report of a kindred with X-linked (or autosomal dominant sex-limited) 46,XY partial gonadal dysgenesis.
The Journal of clinical endocrinology and metabolism 1993;76(5):1248-53.
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1993: Fechner P Y; Marcantonio S M; Jaswaney V; Stetten G; Goodfellow P N; Migeon C J; Smith K D; Berkovitz G D; Amrhein J A; Bard P A
The role of the sex-determining region Y gene in the etiology of 46,XX maleness.
The Journal of clinical endocrinology and metabolism 1993;76(3):690-5.
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1992: Hawkins J R; Taylor A; Goodfellow P N; Migeon C J; Smith K D; Berkovitz G D
Evidence for increased prevalence of SRY mutations in XY females with complete rather than partial gonadal dysgenesis.
American journal of human genetics 1992;51(5):979-84.
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1992: Dover G J; Smith K D; Chang Y C; Purvis S; Mays A; Meyers D A; Sheils C; Serjeant G
Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2.
Blood 1992;80(3):816-24.
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1992: Stine O C; Dover G J; Zhu D; Smith K D
The evolution of two west African populations.
Journal of molecular evolution 1992;34(4):336-44.
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1992: Fechner P Y; Smith K D; Jabs E W; Migeon C J; Berkovitz G D
Partial gonadal dysgenesis in a patient with a marker Y chromosome.
American journal of medical genetics 1992;42(6):807-12.
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1992: Berkovitz G D; Fechner P Y; Marcantonio S M; Bland G; Stetten G; Goodfellow P N; Smith K D; Migeon C J
The role of the sex-determining region of the Y chromosome (SRY) in the etiology of 46,XX true hermaphroditism.
Human genetics 1992;88(4):411-6.
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1992: Moser H W; Moser A B; Smith K D; Bergin A; Borel J; Shankroff J; Stine O C; Merette C; Ott J; Krivit W
Adrenoleukodystrophy: phenotypic variability and implications for therapy.
Journal of inherited metabolic disease 1992;15(4):645-64.
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1990: Stine O C; Smith K D
The estimation of selection coefficients in Afrikaners: Huntington disease, porphyria variegata, and lipoid proteinosis.
American journal of human genetics 1990;46(3):452-8.
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1989: Weissenbach J; Goodfellow P N; Smith K D
Report of the committee on the genetic constitution of the Y chromosome.
Cytogenetics and cell genetics 1989;51(1-4):438-49.
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1988: Weissenbach J; Goodfellow P N; Smith K D
Report of the committee on the genetic constitution of the Y chromosome.
Cytogenetics and cell genetics 1988;49(1-3):129-31.
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1987: Scott A F; Schmeckpeper B J; Abdelrazik M; Comey C T; O'Hara B; Rossiter J P; Cooley T; Heath P; Smith K D; Margolet L
Origin of the human L1 elements: proposed progenitor genes deduced from a consensus DNA sequence.
Genomics 1987;1(2):113-25.
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1987: Smith K D; Young K E; Talbot C C; Schmeckpeper B J
Repeated DNA of the human Y chromosome.
Development (Cambridge, England) 1987;101 Suppl():77-92.
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1985: Schmeckpeper B J; Davis J; Willard H F; Smith K D
An anonymous single-copy X-chromosome RFLP for DXS72 from Xq13-Xq22 [HGM8 provisional no. DXS72].
Nucleic acids research 1985;13(15):5724.
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1985: Burk R D; Ma P; Smith K D
Characterization and evolution of a single-copy sequence from the human Y chromosome.
Molecular and cellular biology 1985;5(3):576-81.
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1985: Burk R D; Szabo P; O'Brien S; Nash W G; Yu L; Smith K D
Organization and chromosomal specificity of autosomal homologs of human Y chromosome repeated DNA.
Chromosoma 1985;92(3):225-33.
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1984: Boyer S H; Dover G J; Serjeant G R; Smith K D; Antonarakis S E; Embury S H; Margolet L; Noyes A N; Boyer M L; Bias W B
Production of F cells in sickle cell anemia: regulation by a genetic locus or loci separate from the beta-globin gene cluster.
Blood 1984;64(5):1053-8.
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1984: Schmeckpeper B J; Scott A F; Smith K D
Transcripts homologous to a long repeated DNA element in the human genome.
The Journal of biological chemistry 1984;259(2):1218-25.
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1984: Boyer S H; Ostrer H; Smith K D; Young K E; Noyes A N
Isolation of cDNA clones for rabbit red cell carbonic anhydrase and catalase: a pilot study directed at isolation of coordinately expressed genes.
Annals of the New York Academy of Sciences 1984;429():324-31.
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1983: Charache S; Dover G; Smith K; Talbot C C; Moyer M; Boyer S
Treatment of sickle cell anemia with 5-azacytidine results in increased fetal hemoglobin production and is associated with nonrandom hypomethylation of DNA around the gamma-delta-beta-globin gene complex.
Proceedings of the National Academy of Sciences of the United States of America 1983;80(15):4842-6.
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1983: Willard H F; Smith K D; Sutherland J
Isolation and characterization of a major tandem repeat family from the human X chromosome.
Nucleic acids research 1983;11(7):2017-33.
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1983: Burk R D; Stamberg J; Young K E; Smith K D
Use of repetitive DNA for diagnosis of chromosomal rearrangements.
Human genetics 1983;64(4):339-42.
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1983: Dover G J; Charache S H; Boyer S H; Talbot C C; Smith K D
5-Azacytidine increases fetal hemoglobin production in a patient with sickle cell disease.
Progress in clinical and biological research 1983;134():475-88.
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1982: Kunkel L M; Smith K D
Evolution of human Y-chromosome DNA.
Chromosoma 1982;86(2):209-28.
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1981: Schmeckpeper B J; Willard H F; Smith K D
Isolation and characterization of cloned human DNA fragments carrying reiterated sequences common to both autosomes and the X chromosome.
Nucleic acids research 1981;9(8):1853-72.
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1981: Scott A F; Phillips J A; Young K E; Kazazian H H; Smith K D; Charache S; Clegg J B
The molecular basis of hemoglobin Grady.
American journal of human genetics 1981;33(1):129-33.
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1981: Panny S R; Scott A F; Smith K D; Phillips J A; Kazazian H H; Talbot C C; Boehm C D
Population heterogeneity of the Hpa I restriction site associated with the beta globin gene: implications for prenatal diagnosis.
American journal of human genetics 1981;33(1):25-35.
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1980: Phillips J A; Vik T A; Scott A F; Young K E; Kazazian H H; Smith K D; Fairbanks V F; Koenig H M
Unequal crossing-over: a common basis of single alpha-globin genes in Asians and American blacks with hemoglobin-H disease.
Blood 1980;55(6):1066-9.
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1980: Phillips J A; Panny S R; Kazazian H H; Boehm C D; Scott A F; Smith K D
Prenatal diagnosis of sickle cell anemia by restriction and endonuclease analysis: HindIII polymorphisms in gamma-globin genes extend test applicability.
Proceedings of the National Academy of Sciences of the United States of America 1980;77(5):2853-6.
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1979: Phillips J A; Scott A F; Smith K D; Young K E; Lightbody K L; Jiji R M; Kazazian H H
A molecular basis for hemoglobin-H disease in American blacks.
Blood 1979;54(6):1439-45.
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1979: Schmeckpeper B J; Smith K D; Dorman B P; Ruddle F H; Talbot C C
Partial purification and characterization of DNA from the human X chromosome.
Proceedings of the National Academy of Sciences of the United States of America 1979;76(12):6525-8.
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1979: Phillips J A; Scott A F; Kazazian H H; Smith K D; Stetten G; Thomas G H
Prenatal diagnosis of hemoglobinopathies by restriction endonuclease analysis: pregnancies at risk for sickle cell anemia and S--O Arab disease.
The Johns Hopkins medical journal 1979;145(2):57-60.
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1979: Kunkel L M; Smith K D; Boyer S H
Organization and heterogeneity of sequences within a repeating unit of human Y chromosome deoxyribonucleic acid.
Biochemistry 1979;18(15):3343-53.
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1979: Hamer D H; Smith K D; Boyer S H; Leder P
SV40 recombinants carrying rabbit beta-globin gene coding sequences.
Cell 1979;17(3):725-35.
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1978: Boyer S H; Scott A F; Kunkel L M; Smith K D
The proportion of all point mutations which are unacceptable: an estimate based on hemoglobin amino acid and nucleotide sequences.
Canadian journal of genetics and cytology. Journal canadien de génétique et de cytologie 1978;20(1):111-37.
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1977: Kunkel L M; Smith K D; Boyer S H; Borgaonkar D S; Wachtel S S; Miller O J; Breg W R; Jones H W; Rary J M
Analysis of human Y-chromosome-specific reiterated DNA in chromosome variants.
Proceedings of the National Academy of Sciences of the United States of America 1977;74(3):1245-9.
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1976: Bacheler L T; Smith K D
Transcription of isolated mouse liver chromatin.
Biochemistry 1976;15(15):3281-90.
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1976: Smith K D; Kunkel L; Boyer S H
Isolation and uses of chromosome-specific reiterated DNA.
Cytogenetics and cell genetics 1976;16(1-5):401-4.
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1976: Smith K D; Kunkel L; Boyer S H
Isolation and uses of chromosome-specific reiterated DNA.
Birth defects original article series 1976;12(7):401-4.
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1972: Schmeckpeper B J; Smith K D
Use of formamide in nucleic acid reassociation.
Biochemistry 1972;11(7):1319-26.
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1972: Tobler H; Smith K D; Ursprung H
Molecular aspects of chromatin elimination in Ascaris lumbricoides.
Developmental biology 1972;27(2):190-203.
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1969: Smith K D; Church R B; McCarthy B J
Template specificity of isolated chromatin.
Biochemistry 1969;8(11):4271-7.
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1967: Smith K D
Genetic control of macromolecular synthesis during development of an ascidian: Ascidia nigra.
The Journal of experimental zoology 1967;164(3):393-405.
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