نتایج جستجو برای: dominant mutation

تعداد نتایج: 404478  

2016
Chantal Depondt Simona Donatello Myriam Rai François Charles Wang Mario Manto Nicolas Simonis Massimo Pandolfo

OBJECTIVE To identify the causative gene mutation in a 5-generation Belgian family with dominantly inherited spinocerebellar ataxia and polyneuropathy, in which known genetic etiologies had been excluded. METHODS We collected DNA samples of 28 family members, including 7 living affected individuals, whose clinical records were reviewed by a neurologist experienced in ataxia. We combined linka...

2015
Blair R. Anderson David N. Howell Karen Soldano Melanie E. Garrett Nicholas Katsanis Marilyn J. Telen Erica E. Davis Allison E. Ashley-Koch

access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Journal: :The Journal of clinical investigation 1992
R A Fleischman

Human piebald trait is an autosomal dominant defect in melanocyte development characterized by patches of hypopigmented skin and hair. Although the molecular basis of piebaldism has been unclear, a phenotypically similar "dominant spotting" of mice is caused by mutations in the murine c-kit protooncogene. In this regard, one piebald case with a point mutation and another with a deletion of c-ki...

Journal: :Journal of bacteriology 2002
Ulrich Strych Michael J Benedik

Alanine racemases are ubiquitous prokaryotic enzymes providing the essential peptidoglycan precursor D-alanine. We present evidence that the enzymes from Pseudomonas aeruginosa and Escherichia coli function exclusively as homodimers. Moreover, we demonstrate that expression of a K35A Y235A double mutation of dadX in E. coli suppresses bacterial growth in a dominant negative fashion.

Journal: :Human molecular genetics 1996
L Ma S Golden L Wu R Maxson

Craniosynostosis, Boston type is an autosomal dominant disorder that results in the premature fusion of calvarial bones and ensuing abnormalities in skull shape. We showed previously that this disorder is tightly linked to the Msx2 homeobox gene on the long arm of chromosome 5, and that affected individuals bear a mutated copy of Msx2. In addition, transgenic mice in which either mutant or wild...

Journal: :journal of dentistry, tehran university of medical sciences 0
najmeh akhlaghi dental research center, department of pediatric dentistry, school of dentistry, isfahan university of medical sciences, isfahan, iran. ali-reza eshghi dental research center, department of pediatric dentistry, school of dentistry, isfahan university of medical sciences, isfahan, iran. mehrnaz mohamadpour dental research center, department of pediatric dentistry, school of dentistry, isfahan university of medical sciences, isfahan, iran.

dentinogenesis imperfecta (di) is a hereditary dentin defect caused by an autosomal dominant mutation in dentin sialophosphoprotein gene. defective dentin development results in discolored teeth that are prone to wear and fracture. early diagnosis and proper treatment are necessary to achieve better functional and esthetic results and minimize nutritional deficiencies and psychosocial distress....

Journal: :Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2015
Jeroen H F de Baaij Eiske M Dorresteijn Eric A M Hennekam Erik-Jan Kamsteeg Rowdy Meijer Karin Dahan Michelle Muller Marinus A van den Dorpel René J M Bindels Joost G J Hoenderop Olivier Devuyst Nine V A M Knoers

BACKGROUND Magnesium (Mg(2+)) is an essential ion for cell growth, neuroplasticity and muscle contraction. Blood Mg(2+) levels <0.7 mmol/L may cause a heterogeneous clinical phenotype, including muscle cramps and epilepsy and disturbances in K(+) and Ca(2+) homeostasis. Over the last decade, the genetic origin of several familial forms of hypomagnesaemia has been found. In 2000, mutations in FX...

Journal: :JAMA neurology 2015
Carlo Rinaldi Thomas Schmidt Alan J Situ Janel O Johnson Philip R Lee Ke-Lian Chen Laura C Bott Rut Fadó George H Harmison Sara Parodi Christopher Grunseich Benoît Renvoisé Leslie G Biesecker Giuseppe De Michele Filippo M Santorelli Alessandro Filla Giovanni Stevanin Alexandra Dürr Alexis Brice Núria Casals Bryan J Traynor Craig Blackstone Tobias S Ulmer Kenneth H Fischbeck

IMPORTANCE The family of genes implicated in hereditary spastic paraplegias (HSPs) is quickly expanding, mostly owing to the widespread availability of next-generation DNA sequencing methods. Nevertheless, a genetic diagnosis remains unavailable for many patients. OBJECTIVE To identify the genetic cause for a novel form of pure autosomal dominant HSP. DESIGN, SETTING, AND PARTICIPANTS We ex...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1952
E D Garber

Although sex-linkage in animals is no longer an unusual phenomenon, its apparent rarity in rodents is noteworthy, especially in the intensively studied house mouse and rat. Hauschka, et al.,1 have presented evidence for a sex-linked lethal altering the sex ratio in the house mouse; Falconer2 recently reported a second sex-linked mutation in the house mouse. A third sex-linked mutation, Bent-tai...

Journal: :Genetics 1972
C Pratt J Gallant

A dominant constitutive mutation of the phoR locus controlling alkaline phosphatase synthesis in Escherichia coli is described. Its phenotype can be explained by the production of a poisonous subunit of the phoR gene product. The phoR gene product is inferred to consist of at least 3 or 4 subunits.

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