Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice.

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Published in Neuroscience on August 27, 2008

Authors

M A Hickey1, A Kosmalska, J Enayati, R Cohen, S Zeitlin, M S Levine, M-F Chesselet

Author Affiliations

1: Department of Neurology, University of California, Los Angeles, David Geffen School of Medicine, Reed Neurological Research Center B114, 710 Westwood Plaza, Los Angeles, CA 90095, USA.

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Social network ties and mortality among the elderly in the Alameda County Study. Am J Epidemiol (1987) 2.69

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Fungal flora of the normal human small and large intestine. N Engl J Med (1969) 2.25

Pre-mRNA splicing and the nuclear matrix. Mol Cell Biol (1987) 2.22

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Plain film diagnosis of the acute abdomen. Emerg Med Clin North Am (1985) 2.11

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Effect of a tumour promoter on myogenesis. Nature (1977) 1.98

Endothelial dysfunction in a murine model of mild hyperhomocyst(e)inemia. J Clin Invest (2000) 1.94

Solubilization of the UDP-glucose:1,4-beta-D-glucan 4-beta-D-glucosyltransferase (cellulose synthase) from Acetobacter xylinum. A comparison of regulatory properties with those of the membrane-bound form of the enzyme. J Biol Chem (1983) 1.94

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Graduates of foreign medical schools: demographic and personal predictors of success on an OSCE-format internship programme entrance examination. Res Med Educ (1988) 1.86

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Reliability and construct validity of a structured technical skills assessment form. Am J Surg (1994) 1.72

Rat insulin-like growth factor II gene. A single gene with two promoters expressing a multitranscript family. J Mol Biol (1986) 1.72

Conditional mutagenesis in mice with heat shock promoter-driven cre transgenes. Mamm Genome (2000) 1.71

Identification of a regulatory region that mediates glucose-dependent induction of the Saccharomyces cerevisiae enolase gene ENO2. Mol Cell Biol (1986) 1.69

Portal venous gas as a complication of ERCP and endoscopic sphincterotomy. Am J Gastroenterol (1995) 1.65

Sedimentation of generalized systems of interacting particles. II. Active enzyme centrifugation--theory and extensions of its validity range. Biopolymers (1975) 1.64

Shared decision making for in-patients with schizophrenia. Acta Psychiatr Scand (2006) 1.63

Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function. Neuroscience (2011) 1.62

Airborne endotoxin associated with industrial-scale production of protein products in gram-negative bacteria. Am Ind Hyg Assoc J (1988) 1.61

Paclitaxel-based chemotherapy in carcinoma of the fallopian tube. Gynecol Oncol (2001) 1.59

Resistance to macrolides in Streptococcus pyogenes in France in pediatric patients. Antimicrob Agents Chemother (2000) 1.57

Benefits of Ophdiat, a telemedical network to screen for diabetic retinopathy: a retrospective study in five reference hospital centres. Diabetes Metab (2009) 1.56

Laparascopic excision of pelvic kidney with single vaginal ectopic ureter. J Pediatr Surg (1997) 1.56

Percolation in directed scale-free networks. Phys Rev E Stat Nonlin Soft Matter Phys (2002) 1.56

Killian-Jamieson diverticula: radiographic findings in 16 patients. AJR Am J Roentgenol (2001) 1.56

Structural family versus psychodynamic child therapy for problematic Hispanic boys. J Consult Clin Psychol (1989) 1.53

Inhaled antibiotics in cystic fibrosis. Lancet (1983) 1.53

Comparison of the 15q deletions in Prader-Willi and Angelman syndromes: specific regions, extent of deletions, parental origin, and clinical consequences. Am J Med Genet (1990) 1.53

Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci (2001) 1.52

Is the period of rapidly declining adult mortality in the United States coming to an end? Am J Public Health (1983) 1.50

The type 2 family: a setting for development and treatment of adolescent type 2 diabetes mellitus. Arch Pediatr Adolesc Med (1999) 1.49

Minimal exon sequence requirements for efficient in vitro splicing of mono-intronic nuclear pre-mRNA. J Biol Chem (1987) 1.48

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Ventricular laceration and cardiac tamponade during laparoscopic Nissen fundoplication. Surg Laparosc Endosc (1996) 1.48

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Crohn's disease arthritis treated with infliximab: an open trial in four patients. J Clin Rheumatol (2001) 1.46

Dopaminergic modulation of NMDA-induced whole cell currents in neostriatal neurons in slices: contribution of calcium conductances. J Neurophysiol (1998) 1.46

Guidelines for estimating the real cost of an objective structured clinical examination. Acad Med (1993) 1.45

Vaccination against meningococcus C. vaccinal coverage in the French target population. Med Mal Infect (2013) 1.45

Increasing trends in the use of breast-conserving surgery in California. Am J Public Health (2000) 1.44

Normal viral and bacterial flora of the human small and large intestine. N Engl J Med (1966) 1.44

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Sexual agglutinins from the Chlamydomonas flagellar membrane. Partial purification and characterization. J Biol Chem (1982) 1.42

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An objective structured clinical examination for the licentiate of the Medical Council of Canada: from research to reality. Acad Med (1993) 1.41

Undifferentiated (embryonal) sarcoma of the liver: pathologic basis of imaging findings in 28 cases. Radiology (1997) 1.40

Visualization of areae gastricae on double-contrast upper gastrointestinal radiography: relationship to age of patients. AJR Am J Roentgenol (2001) 1.39

[Meningeal myelomatosis. Presentation of a case]. Sangre (Barc) (1992) 1.38

Analysis of excess lung cancer risk in short-term employees. Am J Epidemiol (1988) 1.38

The hot clot sign. A new finding in deep venous thrombosis on bone scintigraphy. Clin Nucl Med (1990) 1.38

[Treatment of Streptococcus pneumoiae meningitis in infants]. Arch Pediatr (1994) 1.37

In vitro killing activities of antibiotics at clinically achievable concentrations in cerebrospinal fluid against penicillin-resistant Streptococcus pneumoniae isolated from children with meningitis. Antimicrob Agents Chemother (1994) 1.37

Factors contributing to physicians' success in the advanced trauma life support program in Israel. Isr Med Assoc J (1999) 1.36

Null mutation of the murine ATP7B (Wilson disease) gene results in intracellular copper accumulation and late-onset hepatic nodular transformation. Hum Mol Genet (1999) 1.36

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A comparative analysis of the costs of administration of an OSCE (objective structured clinical examination). Acad Med (1994) 1.34

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Behavioral effects of dopaminergic agonists in transgenic mice overexpressing human wildtype alpha-synuclein. Neuroscience (2006) 1.32

Microbial intestinal flora in acute diarrheal disease. JAMA (1967) 1.30

The screw gene encodes a ubiquitously expressed member of the TGF-beta family required for specification of dorsal cell fates in the Drosophila embryo. Genes Dev (1994) 1.28

Impact of antimicrobial therapy on nasopharyngeal carriage of Streptococcus pneumoniae, Haemophilus influenzae, and Branhamella catarrhalis in children with respiratory tract infections. Clin Infect Dis (2000) 1.27

Meckel's enteroliths: clinical, radiologic, and pathologic findings. AJR Am J Roentgenol (1996) 1.27

Ventilatory and diffusion abnormalities in potential heart transplant recipients. Chest (1990) 1.25

Guinea pig preproinsulin gene: an evolutionary compromise? Proc Natl Acad Sci U S A (1984) 1.24

Cryoglobulins. III. Further studies on the nature, incidence, clinical, diagnostic, prognostic, and immunopathologic significance of cryoproteins in renal disease. Q J Med (1975) 1.24

Functional analysis of mononuclear cells infiltrating into tumors: lysis of autologous human tumor cells by cultured infiltrating lymphocytes. Cancer Res (1987) 1.23

Murine Hox-1.7 homeo-box gene: cloning, chromosomal location, and expression. Mol Cell Biol (1987) 1.23

Metformin and digestive disorders. Diabetes Metab (2011) 1.22

Facilitated glutamatergic transmission in the striatum of D2 dopamine receptor-deficient mice. J Neurophysiol (2001) 1.21

Changes in apparent body orientation and sensory localization induced by vibration of postural muscles: vibratory myesthetic illusions. Aviat Space Environ Med (1979) 1.20

Pregnancy and idiopathic autoimmune haemolytic anaemia: a prospective study during 6 months gestation and 3 months post-partum. Br J Haematol (1973) 1.19