Published in Can J Neurol Sci on January 01, 2009
Blood cells from Friedreich ataxia patients harbor frataxin deficiency without a loss of mitochondrial function. Mitochondrion (2010) 0.96
Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models. PLoS One (2014) 0.87
Excision of Expanded GAA Repeats Alleviates the Molecular Phenotype of Friedreich's Ataxia. Mol Ther (2015) 0.87
Delay in apoptosome formation attenuates apoptosis in mouse embryonic stem cell differentiation. J Biol Chem (2014) 0.83
Frataxin silencing inactivates mitochondrial Complex I in NSC34 motoneuronal cells and alters glutathione homeostasis. Int J Mol Sci (2014) 0.78
Functional characterization of Friedreich ataxia iPS-derived neuronal progenitors and their integration in the adult brain. PLoS One (2014) 0.77
Gene expression profiling of mitochondrial oxidative phosphorylation (OXPHOS) complex I in Friedreich ataxia (FRDA) patients. PLoS One (2014) 0.77
Friedreich Ataxia: current status and future prospects. Cerebellum Ataxias (2017) 0.75
Novel Point Mutations in Frataxin Gene in Iranian Patients with Friedreich's Ataxia. Iran J Child Neurol (2014) 0.75
Liver Growth Factor (LGF) Upregulates Frataxin Protein Expression and Reduces Oxidative Stress in Friedreich's Ataxia Transgenic Mice. Int J Mol Sci (2016) 0.75
Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia. Biol Open (2016) 0.75
Mitochondrial genomes of extinct aurochs survive in domestic cattle. Curr Biol (2008) 2.14
KIF1A, an axonal transporter of synaptic vesicles, is mutated in hereditary sensory and autonomic neuropathy type 2. Am J Hum Genet (2011) 1.66
Four novel C20orf54 mutations identified in Brown-Vialetto-Van Laere syndrome patients. J Hum Genet (2012) 1.57
Mitochondrial genomes from modern horses reveal the major haplogroups that underwent domestication. Proc Natl Acad Sci U S A (2012) 1.55
Mitochondrial DNA signals of late glacial recolonization of Europe from near eastern refugia. Am J Hum Genet (2012) 1.49
Sodium current and potassium transient outward current genes in Brugada syndrome: screening and bioinformatics. Can J Cardiol (2012) 1.43
Hexosamine biosynthetic pathway mutations cause neuromuscular transmission defect. Am J Hum Genet (2011) 1.39
Prevalence of human T-lymphotropic virus type 1 among blood donors from Mashhad, Iran. J Clin Microbiol (2003) 1.31
Homozygous missense mutation in fibulin-5 in an Iranian autosomal recessive cutis laxa pedigree and associated haplotype. J Invest Dermatol (2006) 1.22
Ancient migratory events in the Middle East: new clues from the Y-chromosome variation of modern Iranians. PLoS One (2012) 1.03
Distinguishing the co-ancestries of haplogroup G Y-chromosomes in the populations of Europe and the Caucasus. Eur J Hum Genet (2012) 1.03
High prevalence of AZFb microdeletion in Iranian patients with idiopathic non-obstructive azoospermia. Indian J Med Res (2010) 1.00
Comprehensive study of mtDNA among Southwest Asian dogs contradicts independent domestication of wolf, but implies dog-wolf hybridization. Ecol Evol (2011) 0.99
MTHFR polymorphisms and breast cancer risk. Arch Med Sci (2011) 0.99
Effects of miglustat on stabilization of neurological disorder in niemann-pick disease type C: Iranian pediatric case series. J Child Neurol (2012) 0.96
Mitochondrial complex I deficiency and ATP/ADP ratio in lymphocytes of amyotrophic lateral sclerosis patients. Neurol Res (2012) 0.95
Vitamin D receptor homozygote mutant tt and bb are associated with susceptibility to pulmonary tuberculosis in the Iranian population. Int J Infect Dis (2009) 0.93
Enhancement of a bacterial laccase thermostability through directed mutagenesis of a surface loop. Enzyme Microb Technol (2011) 0.91
Downregulation of miR-34a in breast tumors is not associated with either p53 mutations or promoter hypermethylation while it correlates with metastasis. Med Oncol (2013) 0.91
Complex I deficiency in Persian multiple sclerosis patients. J Neurol Sci (2006) 0.89
Association between calcium-sensing receptor gene polymorphisms and recurrent calcium kidney stone disease: a comprehensive gene analysis. Scand J Urol Nephrol (2010) 0.89
High rate of mutation in mitochondrial DNA displacement loop region in human colorectal cancer. Dis Colon Rectum (2009) 0.89
Association between trinucleotide CAG repeats of the DNA polymerase gene (POLG) with age of onset of Iranian Friedreich's ataxia patients. Neurol Sci (2008) 0.89
The influence of insertion of a critical residue (Arg356) in structure and bioluminescence spectra of firefly luciferase. J Biol Chem (2006) 0.89
Effect of skin thickness on sensory nerve action potential amplitude. Clin Neurophysiol (2008) 0.88
The POLG Gene Polymorphism in Iranian Varicocele-Associated Infertility Patients. Iran J Basic Med Sci (2012) 0.87
Design and introduction of a disulfide bridge in firefly luciferase: increase of thermostability and decrease of pH sensitivity. Photochem Photobiol Sci (2010) 0.87
Mitochondrial tRNALeu/Lys and ATPase 6/8 gene variations in spinocerebellar ataxias. Neurodegener Dis (2008) 0.86
Beta-Thalassemia in Iran: new insight into the role of genetic admixture and migration. ScientificWorldJournal (2012) 0.86
Analysis of mitochondrial ND1 gene in human colorectal cancer. J Res Med Sci (2011) 0.86
Huntington's disease and mitochondrial DNA deletions: event or regular mechanism for mutant huntingtin protein and CAG repeats expansion?! Cell Mol Neurobiol (2007) 0.86
RAD51 polymorphisms and breast cancer risk. Mol Biol Rep (2012) 0.86
A novel mutation in the transactivation-regulating domain of the androgen receptor in a patient with azoospermia. J Androl (2010) 0.85
Design of disulfide bridge as an alternative mechanism for color shift in firefly luciferase and development of secreted luciferase. Photochem Photobiol Sci (2011) 0.85
Preparation, characterization, and efficient transfection of cationic liposomes and nanomagnetic cationic liposomes. Int J Nanomedicine (2011) 0.85
Kinetic properties of extracted lactate dehydrogenase and creatine kinase from mouse embryonic stem cell- and neonatal-derived cardiomyocytes. J Biochem Mol Biol (2006) 0.85
Investigation on mitochondrial tRNA(Leu/Lys), NDI and ATPase 6/8 in Iranian multiple sclerosis patients. Cell Mol Neurobiol (2007) 0.85
A novel mitochondrial heteroplasmic C13806A point mutation associated with Iranian Friedreich's ataxia. Cell Mol Neurobiol (2008) 0.84
Nanofiber-based polyethersulfone scaffold and efficient differentiation of human induced pluripotent stem cells into osteoblastic lineage. Mol Biol Rep (2013) 0.84
Design and characterization of novel trypsin-resistant firefly luciferases by site-directed mutagenesis. Protein Eng Des Sel (2009) 0.84
How benzene and its metabolites affect human marrow derived mesenchymal stem cells. Toxicol Lett (2012) 0.84
Comparative proteomic analysis of mouse embryonic stem cells and neonatal-derived cardiomyocytes. Biochem Biophys Res Commun (2006) 0.84
Delta mtDNA4977 is more common in non-tumoral cells from gastric cancer sample. Arch Med Res (2006) 0.83
Molecular cloning, sequence analysis, and expression of a cDNA encoding the luciferase from the glow-worm, Lampyris turkestanicus. Biochem Biophys Res Commun (2004) 0.83
Purification and characterization of a thermostable phytate resistant alpha-amylase from Geobacillus sp. LH8. Int J Biol Macromol (2009) 0.83
Validation of the Persian version of the 40-item amyotrophic lateral sclerosis assessment questionnaire. Iran J Neurol (2013) 0.83
Repeat expansion in C9ORF72 is not a major cause of amyotrophic lateral sclerosis among Iranian patients. Neurobiol Aging (2013) 0.82
Do haplogroups H and U act to increase the penetrance of Alzheimer's disease? Cell Mol Neurobiol (2006) 0.82
BAX pro-apoptotic gene alterations in repeated pregnancy loss. Arch Med Sci (2011) 0.82
No mitochondrial DNA deletions but more D-loop point mutations in repeated pregnancy loss. J Assist Reprod Genet (2010) 0.82
Accumulation of mitochondrial genome variations in Persian LQTS patients: a possible risk factor? Cardiovasc Pathol (2009) 0.82
Red blood cell ATP/ADP & nitric oxide: The best vasodilators in diabetic patients. J Diabetes Metab Disord (2012) 0.82
Investigation of tRNA(Leu/Lys) and ATPase 6 genes mutations in Huntington's disease. Cell Mol Neurobiol (2008) 0.82
Association of fibroblast growth factor (FGF-21) as a biomarker with primary mitochondrial disorders, but not with secondary mitochondrial disorders (Friedreich Ataxia). Mol Biol Rep (2013) 0.81
A novel PANK2 gene mutation in a Persian boy: the first report from Iran. Clin Neurol Neurosurg (2012) 0.81
Four cases of brown-vialetto-van laere syndrome from Iran: Clinical and electrophysiologic findings. Iran J Neurol (2011) 0.81
Silibinin inhibits invasive properties of human glioblastoma U87MG cells through suppression of cathepsin B and nuclear factor kappa B-mediated induction of matrix metalloproteinase 9. Anticancer Drugs (2010) 0.81
Expression analysis and purification of human recombinant tissue type plasminogen activator (rt-PA) from transgenic tobacco plants. Prep Biochem Biotechnol (2011) 0.81
Limited proteolysis of luciferase as a reporter in nanosystem biology: a comparative study. Photochem Photobiol (2009) 0.80
Analysis of HLA DR2&DQ6 (DRB1*1501, DQA1*0102, DQB1*0602) haplotypes in Iranian patients with multiple sclerosis. Cell Mol Neurobiol (2008) 0.80
Establishment and characterization of two human breast carcinoma cell lines by spontaneous immortalization: Discordance between Estrogen, Progesterone and HER2/neu receptors of breast carcinoma tissues with derived cell lines. Cancer Cell Int (2012) 0.80
Design and development of a whole-cell luminescent biosensor for detection of early-stage of apoptosis. Biosens Bioelectron (2012) 0.80
Wilson's disease: a great masquerader. Eur Neurol (2006) 0.80
Effect of charge distribution in a flexible loop on the bioluminescence color of firefly luciferases. Biochemistry (2009) 0.80
Genetic analysis and SOD1 mutation screening in Iranian amyotrophic lateral sclerosis patients. Neurobiol Aging (2012) 0.80
Identification of mutation in NPC2 by exome sequencing results in diagnosis of Niemann-Pick disease type C. Mol Genet Metab (2013) 0.80
Evaluation of point mutations in dystrophin gene in Iranian Duchenne and Becker muscular dystrophy patients: introducing three novel variants. J Genet (2016) 0.80
Design, engineering and preparation of a multi-domain fusion vector for gene delivery. Int J Pharm (2012) 0.79
The effect of hot-tub therapy on serum Hsp70 level and its benefit on diabetic rats: a preliminary report. Int J Hyperthermia (2010) 0.79
Comparison of the effects of L: -carnitine and alpha-tocopherol on acute ureteral obstruction-induced renal oxidative imbalance and altered energy metabolism in rats. Urol Res (2009) 0.79
Cloning, sequencing, expression and structural investigation of mnemiopsin from Mnemiopsis leidyi: an attempt toward understanding Ca2+-regulated photoproteins. Protein J (2011) 0.79
Novel nucleotide changes in mutational analysis of mitochondrial 12SrRNA gene in patients with nonsyndromic and aminoglycoside-induced hearing loss. Mol Biol Rep (2012) 0.79
Mitochondrial D-loop variation in leber hereditary neuropathy patients harboring primary G11778A, G3460A, T14484C mutations: J and W haplogroups as high-risk factors. Arch Med Res (2006) 0.79
cDNA cloning, expression and homology modeling of a luciferase from the firefly Lampyroidea maculata. J Biochem Mol Biol (2006) 0.79
Chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus. J Res Med Sci (2013) 0.79
L-carnitine improves oxidative stress and suppressed energy metabolism but not renal dysfunction following release of acute unilateral ureteral obstruction in rat. Neurourol Urodyn (2011) 0.79
Site-directed mutagenesis of firefly luciferase: implication of conserved residue(s) in bioluminescence emission spectra among firefly luciferases. Biochem J (2008) 0.79
Investigation of the Mitochondrial ATPase 6/8 and tRNA(Lys) Genes Mutations in Autism. Cell J (2012) 0.79
Tumoral cell mtDNA approximately 8.9 kb deletion is more common than other deletions in gastric cancer. Arch Med Res (2006) 0.79
Static magnetic field of 6 mT induces apoptosis and alters cell cycle in p53 mutant Jurkat cells. Electromagn Biol Med (2013) 0.79
T4216C mutation in NADH dehydrogenase I gene is associated with recurrent pregnancy loss. Mitochondrial DNA (2013) 0.79
Delay expression of limonoid UDP-glucosyltransferase makes delayed bitterness in citrus. Biochem Biophys Res Commun (2008) 0.79
Split-luciferase complementary assay: applications, recent developments, and future perspectives. Anal Bioanal Chem (2014) 0.78
Apolipoprotein e gene polymorphism in Iranian coronary atherosclerosis patients candidate for coronary artery bypass graft. Iran J Basic Med Sci (2013) 0.78
Luciferase protection against proteolytic degradation: a key for improving signal in nano-system biology. J Biotechnol (2009) 0.78
IL7R and RAG1/2 genes mutations/polymorphisms in patients with SCID. Iran J Allergy Asthma Immunol (2011) 0.78
Therapeutic efficacy of silibinin on human neuroblastoma cells: Akt and NF-κB expressions may play an important role in silibinin-induced response. Neurochem Res (2012) 0.78
Three novel mutations in Iranian patients with Tay-Sachs disease. Iran Biomed J (2014) 0.78
Relationship between stability and bioluminescence color of firefly luciferase. Photochem Photobiol Sci (2010) 0.78
Diagnostic algorithm for identification of individuals with hereditary predisposition to breast cancer. Lik Sprava (2008) 0.78
Usage of mitochondrial D-loop variation to predict risk for Huntington disease. Mitochondrial DNA (2014) 0.78
A unique EF-hand motif in mnemiopsin photoprotein from Mnemiopsis leidyi: implication for its low calcium sensitivity. Biochem Biophys Res Commun (2011) 0.78
Differentiation of human skin-derived precursor cells into functional islet-like insulin-producing cell clusters. In Vitro Cell Dev Biol Anim (2015) 0.78