1. Mitochondrial Genetics How does a murine cell cope with loss of mitochondrial DNA? The How is the mitochondrial transcription machinery regulated? How http://www.mednut.ki.se/research/ngl/mito.html

How does a murine cell cope with loss of mitochondrial DNA? The answer is different depending on cell type, but the end is always the same; cell death. The group is situated at the Department of Medical Nutrition at Karolinska Institutet in Stockholm, the beautiful capital of Sweden. From being rather small, the team has expanded significantly during the last couple of years and now consists of seven postdoctoral fellows, five graduate students, one administrator and one technical assistant. The research carried out in the laboratory is very diverse, but the main focus is on mitochondrial transcription and what happens when it is impaired. We have to date primarily worked with the nuclear encoded mitochondrial transcription factor A, Tfam, and what happens when this protein is lacking. Tfam is essential for mtDNA maintenance and loss of the Tfam protein leads to a complete loss of mtDNA. Oxidative phosphorylation, OXPHOS; is depending on mitochondrial encoded subunits to function, so the mitochondria are not able to produce ATP if mtDNA is gone. We have knocked out the Tfam gene from the entire mouse as well as in individual tissues such as skeletal muscle, heart, pancreas and brain. In all instances the affected cells cannot survive without the Tfam protein. For how long depends on the affected tissue and how early the knockout occurs.

2. ERIC A. SCHON mitochondrial genetics AND THE MOLECULAR BASIS OF HUMAN MITOCHONDRIAL DISEASE Mitochondria are unique among the constituents of the eukaryotic cell in that http://cpmcnet.columbia.edu/dept/genetics/faculties/Schon.html

ERIC A. SCHON, Ph.D. Professor MITOCHONDRIAL GENETICS AND THE MOLECULAR BASIS OF HUMAN MITOCHONDRIAL DISEASE: Mitochondria are unique among the constituents of the eukaryotic cell in that they are semi-autonomous organelles that contain their own genetic machinery. As such, they operate under the dual genetic controls of nuclear DNA (nDNA) and mitochondrial DNA (mtDNA). Mitochondrial genetics differs markedly from mendelian genetics, because first, mitochondria are inherited exclusively from the mother, and second, there are hundreds or thousands of mitochondria (and mtDNAs) per cell. In addition, organellar division and mtDNA replication are stochastic processes unrelated to the cell cycle, and mtDNA gene organization, DNA replication, RNA transcription, and protein translation all have a prokaryotic "look" about them. This latter feature is no surprise, given that mitochondria were once bacteria that were taken up by the proto-eukaryotic cell early in evolution. Biochemically, the most relevant aspect of mitochondrial function is the production of oxidative energy via the respiratory chain and oxidative phosphorylation. Mitochondrial diseases have turned out to be equally unusual. There are maternally-inherited, mendelian-inherited, sporadic, and even environmentally induced mitochondrial disorders, most of which are either severely debilitating or fatal. We are studying the molecular basis of a number of these diseases, most of which are heteroplasmic (i.e. both mutant and wild-type mtDNAs coexist in varying proportions in the same patient), using a novel tissue culture system called "r

3. Basic Genetics what this author defines as "mitochondrial disease". Herein, mitochondrial disease refers to any illness has a great explanation of mitochondrial genetics and can be found at http://www.umdf.org/mitodisease/genetics.html

The Genetics of Mitochondrial Disease Autosomal Recessive Inheritance Maternal Inheritance X-linked Recessive Inheritance Autosomal Dominant Inheritance ... Sporadic Introduction and Definition Unfortunately, the multiple diseases classified as mitochondrial disorders are inherited in different manners. In fact, nearly every inheritance "model" known has been demonstrated to occur in mitochondrial disease. However, most mitochondrial disorders known to date are inherited in either an autosomal recessive or maternal manner. The model of inheritance is important in that it can be helpful in answering the following questions: Are other family members, either existing or not yet born, at risk for developing mitochondrial disease? What is the risk (percentage)? When will other affected relatives become ill? Will other affected relatives be as sick as my child/myself? Possibly even sicker? What kinds of problems/diseases might other affected relatives suffer from?

4. Mitochondrial Genetics Publications Genetic modification of survival in tissuespecific knockout mice with mitochondrial cardiomyopathy. Proc Natl Acad Sci US A. 2000 Mar 28;97(7)3467-72. http://www.mednut.ki.se/research/ngl/pub.html

List of publications Rantanen A, Gaspari M, Falkenberg M, Gustafsson CM, Larsson NG. Characterization of the mouse genes for mitochondrial transcription factors B1 and B2. Mammalian Genome 2003 14(1): 1-6 Graff C, Bui TH, Larsson NG. Wredenberg A, Wibom R, Wilhelmsson H, Graff C, Wiener HH, Burden SJ, Oldfors A, Westerblad H, Larsson NG. ... . A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts. PNAS. 2004 Mar 2;101(9):3136-41.

5. Genetics & Development: Faculty cancercenter.columbia.edu Eric A. Schon Molecular genetics of neurological and neuromuscular disorders with focus on mitochondrial genetics and diseases. http://cpmcnet.columbia.edu/dept/genetics/faculty.html

Genetics and Development Faculty Training Program Department Seminars ... Columbia University Health Sciences Department of Genetics and Development Arthur Bank Regulation of human globin gene expression in hematopoietic cells; gene transfer in animals. ab13@columbia.edu Timothy H. Bestor DNA methyltransferases; epigenetic effects in gene expression. thb12@columbia.edu Marian Carlson Regulation of the Snf1/AMPK family of metabolic stress response kinases and transcriptional responses to stress in yeast. mbc1@columbia.edu Angela M. Christiano Molecular basis of inherited skin and hair disorders in humans and mice. amc65@columbia.edu Franklin D. Costantini Molecular genetics of mammalian development. fdc3@columbia.edu Riccardo Dalla-Favera Molecular genetics of cancer; molecular pathogenesis of lymphoma and leukemia; genetic control in lymphoid tissue development. rd10@columbia.edu Argiris Efstratiadis Growth factors in mammalian development; mouse models of tumorigenic pathways; stem cells in normalcy and malignancy. arg@cancercenter.columbia.edu

6. Entrez PubMed mitochondrial genetics and hearing loss the missing link between genotype and phenotype. FischelGhodsian N. Ahmanson Department http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Retrieve&dopt=Citati

7. Entrez PubMed mitochondrial genetics of mammalian cells a mouse antimycinresistant mutant with a probable alteration of cytochrome b. Howell N, Huang P, Kelliher K, Ryan ML http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Retrieve&dopt=Citati

8. Mitochondrial Genetics - MavicaNET KulturVidenskabLife ScienceGenetics mitochondrial genetics. This category is not edited 15. mitochondrial genetics. Sites total 5 http://www.mavicanet.com/directory/dan/7565.html

selCatSelAlt="Deselect category"; selCatDesAlt="Select category"; selSitSelAlt="Deselect site"; selSitDesAlt="Select site"; STELLA ART GALLERY Andy Warhol Tom Wesselmann Jean-Michel Basquiat MavicaNET - Flersproglig Søgekatalog MavicaNet Lite - Light version Katalog Belarusian Bulgarian Croatian Czech Danish Dutch English Estonian Finnish French German Greek Hungarian Icelandic Irish Italian Norwegian Polish Portuguese Romanian Russian Serbian (cyr.) Serbian (lat.) Slovak Spanish Swedish Turkish Ukrainian Kultur Videnskab Life Science Genetics Mitochondrial Genetics This category is not edited. Ever thought of becoming an editor Sites Sister categories ... Animal Genetics Basic of Genetics Famous Geneticists Genetic Engineering Genetics Journals Genetics of Microorganisms Genetics WWW Resources Molecular Genetics Plant Genetics Population Genetics Sites No filters selected ... Web Resources News Job Education Personalia Organizations References and Indices Humor and entertainment Publications Chats and Forums Shopping Mitochondrial Genetics Sites total: 5 Categories No Sorting Quality Title Rating Language Last Edit Time Mitomap - English URL: http://www.gen.emory.edu/mitomap.html

9. Mitochondrial Genetics Laboratory current projects. Mitochondrial Oxidative Phosphorylation. more pages. RESEARCH HIGHLIGHTS. Frataxin is a novel mitochondrial ironstorage protein. http://mayoresearch.mayo.edu/mayo/research/isayalab/

Medical Services Jobs News current projects Mitochondrial Oxidative Phosphorylation more pages Lab Directory Lab Photos Publications Mitochondrial Genetics Lab ... Printable Version RESEARCH HIGHLIGHTS Frataxin is a novel mitochondrial iron-storage protein. Frataxin is a highly conserved mitochondrial protein that plays a critical role in iron homeostasis. A deficiency of frataxin is the primary cause of Friedreich ataxia (FRDA) ( http://www.ncbi.nlm.nih.gov/Omim ), an autosomal recessive degenerative disease. Abnormal cellular iron distribution and increased oxidative damage are associated with frataxin defects in yeast as well as FRDA but the underlying pathogenic mechanism is unknown. We have shown that ferrous iron stimulates self-assembly of yeast frataxin (Yfh1p) into regular multimers capable of storing as many as 3,000 atoms of iron in soluble form ( Adamec et al. 2000 More... Grazia Isaya M.D., Ph.D. isaya@mayo.edu CONTACT US Grazia Isaya, M.D., Ph.D. Mayo Clinic 200 First Street, SW Stabile 7-52 Rochester, MN 55905 PHONE: (507)266-0110 FAX: (507)266-9315 FEATURED PROJECT Mitochondrial Oxidative Phosphorylation More about Mitochondrial Oxidative Phosphorylation FUTURE PROJECTS What are the functional domains of frataxin?

10. Mitochondrial Genetics - MavicaNET KulttuuriTiedeBiologiaGenetics mitochondrial genetics. This category is not edited 15. mitochondrial genetics. Sites total 5 http://www.mavicanet.com/directory/fin/7565.html

selCatSelAlt="Deselect category"; selCatDesAlt="Select category"; selSitSelAlt="Deselect site"; selSitDesAlt="Select site"; STELLA ART GALLERY Andy Warhol Tom Wesselmann Jean-Michel Basquiat MavicaNET - Monikielinen Hakuluettelo MavicaNet Lite - Light version Luettelo Belarusian Bulgarian Croatian Czech Danish Dutch English Estonian Finnish French German Greek Hungarian Icelandic Irish Italian Norwegian Polish Portuguese Romanian Russian Serbian (cyr.) Serbian (lat.) Slovak Spanish Swedish Turkish Ukrainian Kulttuuri Tiede Biologia Genetics Mitochondrial Genetics This category is not edited. Ever thought of becoming an editor Sites Sister categories ... Animal Genetics Basic of Genetics Famous Geneticists Genetic Engineering Genetics Journals Genetics of Microorganisms Genetics WWW Resources Molecular Genetics Plant Genetics Population Genetics Sites No filters selected ... Web ressurssit Uutiset Työ Koulusivistys Personalia Järjestöt Suositukset ja hakemistot Huumori ja huvit Julkaisut Juutelut ja foorumit Ostokset Mitochondrial Genetics Sites total: 5 Categories No Sorting Quality Title Rating Language Last Edit Time Mitomap - English URL: http://www.gen.emory.edu/mitomap.html

11. LTU: Microbiology: Dictyostelium Mitochondrial Genetics Laboratory Dr. Christian Barth Welcome to the Dictyostelium mitochondrial genetics Page. Location mitochondrial genetics Laboratory Department http://www.latrobe.edu.au/microbiology/barth.html

About LTU Faculties Campuses Research ... Our Research Mitochondrial Genetics Our Staff Seminars Site Map Web Links Dr. Christian Barth Welcome to the Dictyostelium Mitochondrial Genetics Page Location: Mitochondrial Genetics Laboratory Department of Microbiology La Trobe University, Victoria, 3086 AUSTRALIA Contact Information: Office Phone: Lab Phone: Fax: E-mail: C.Barth@latrobe.edu.au Click here to see Dr Christian Barth's CV Research Interests: Mitochondria are found in almost all eukaryotic cells. They are the powerhouse of the cell, generating energy in form of ATP, and have other important functions in haem and lipid biosynthesis, intracellular signal transduction and programmed cell death. According to the endosymbiotic hypothesis Although the number of proteins still encoded in the mitochondrial genome is relatively small, the organellar genome has to be maintained, replicated and transcribed, and the encoded gene products have to be synthesised on ribosomes in the mitochondrial matrix. The genetic processes involved are quite different from those in the nucleus and in the cytoplasm of the eukaryotic host - in fact they are strikingly similar to the genetic processes found in bacteria. However, the proteins that mediate mitochondrial replication, transcription and protein synthesis are now all nuclear-encoded.

12. Mitochondrial Genetics Innovations And Patents mitochondrial genetics Innovations and Patents. More information on mitochondrial genetics and mitochondrial genetics Research References. http://www.air.xq23.com/energy_science_resources/Mitochondrial_Genetics.html

Mitochondrial Genetics Innovations and Patents © 2002, XQ23.COM Research (air.xq23.com) Who were the Great Minds Absolute Zero Acid Rain Acoelomates ... HOME KEY SITES: PRIME-RADIANT FUTURE'S EDGE Future Books Educational Priorities THE LUMINARIES SCIENCE FICTION ARCHIVES: FIRST PILLAR kurt vonnegut arthur c clarke david brin ... Dialysis More information on: Mitochondrial Genetics and Mitochondrial Genetics Research References. Recent U.S. patents related to Mitochondrial Genetics: 5,989,557: Process for extracting polyphenol fractions of tea and compositions produced therewith : scattered data using color to represent values of a categorical variable 6,373,131: TBGA semiconductor package 6,372,842: Methods of using an aqueous composition containing a water-soluble or water-dispersible synthetic polymer and resultant compositions formed thereof 6,372,838: Fine latex and seed method of making 6,372,833: Fluorocarbon thermoplastic random copolymer composition curable at low temperatures 6,372,822: Methods and apparatus for producing and treating novel elastomer composites 6,372,700: Fluorinated solvent compositions containing ozone

13. DOE Document - Mitochondrial Genetics Principles And Practice An improved diagnostic protocol for detecting mitochondrial DNA mutations and the resulting oxidative pohphorylation disorders is presented.^The protocol focuses on six elements (1) clinical cause of chronic degenerative diseases. mitochondrial genetics Principles and practice http://rdre1.inktomi.com/click?u=http://www.osti.gov/energycitations/product.bib

14. Recent Publications By NIST Authors Levin, B. C. Tully, L. A. (BIOTECHNOLOGY DIVISION 831) Human mitochondrial genetics Biotechnology Genetic Engineering Reviews - August 01, 2000 The field http://ois.nist.gov/nistpubs/technipubs/recent/search.cfm?dbibid=4305

15. Genomic Disorders: Mutation Research, Genetic Disease Genomic Disorders Research Centre. mitochondrial genetics. http://www.genomic.unimelb.edu.au/pmito.html

Genomic Disorders Research Centre Mitochondrial Genetics About Us Contact Us Support Us Research News Education Resource Centre Seminars Personnel Conferences Workshops Related Links Site Map Dr. Ian Trounce joined the Centre in 1997 from the world's leading laboratory in this area. Numerous adult and children's disorders are caused by faults in the mitochondria and this work involves important collaborators across Melbourne. Using cell biology, genetic and biochemical approaches, we aim to improve our understanding of the variable clinical features of mitochondrial diseases to investigate mitochondrial function in neurodegenerative diseases, and create robust mtDNA mutation detection methods to define levels of somatic mtDNA mutations. Mechanisms of pathogenesis in mtDNA diseases Background: A major ongoing interest of the group has been to help define the cellular mechanisms underlying the extremely variable phenotypes seen in the mtDNA diseases. In particular we are interested in understanding the contributions of decreased ATP supply and increased oxidative stress, to pathology. We use cell culture methods including the transmission of mtDNAs of interest between cell lines (cybrid transfer), together with biochemical definition of key mitochondrial functions, especially oxidative phosphorylation and apoptosis. A major current project is to produce unique mouse models of mtDNA disease by introducing mtDNAs from different muridae species into mouse embryonic stem cells. In January 2001 we succeeded in producing chimeric animals from our transmitochondrial stem cells.

16. Mitochondrial Genetics mitochondrial genetics Mitochondria (mitochondrion, singular) are little organelles found in the cytoplasm of most cells. They are http://schools.sd68.bc.ca/ed611/phyllida/Mitochon_gen.htm

Mitochondrial Genetics Mitochondria (mitochondrion, singular) are little organelles found in the cytoplasm of most cells. They are often referred to as the "power houses" of the cell because they are the sites of ATP (chemical energy) formation. Structually, mitochondria are bean shaped and are bounded by two membranes. The inner membrane is folded into cristae which is the site of ATP formation. Mitochondria possess their own circular (usually) DNA molecule in multiple copies. Mitochondrial DNA can be differentiated from nuclear DNA because it has a different base (examples of nitrogenous bases are adenine, thymine, cytosine and guanine) composition. Experiments have shown that the genes on mitochondrial DNA govern the normal structure and function of these organelles.

17. J. Piskur - Mitochondrial Genetics Of The Genus Saccharomyces . 93 28 09 mitochondrial genetics of the genus Saccharomyces. J. Piskur, C. Groth, M. Manuel and RF Petersen . Department of Genetics http://www.yeastgenome.org/yeast96/f3028.html

Yeast Genetics and Molecular Biology 1996 Madison, Wisconsin August 1996 Name: j. piskur . Mailing Address: Microbiology, DTU, Bldg. 301, 2800 Lyngby , Denmark Email Address: jurecph@biobase.dk Phone and Fax numbers: Mitochondrial genetics of the genus Saccharomyces J. Piskur, C. Groth, M. Manuel and R.F. Petersen . Department of Genetics, University of Copenhagen, Oester Farimagsgade 2A, 1353 Copenhagen K, Denmark The Saccharomyces cerevisiae mitochondrial genome (mtDNA) consists of coding regions, introns and intergenic regions. The intergenic sequences represent two-thirds of the genome and are made up of adenine-thymine streches and numerous guanosine-cytosine (G-C) clusters. These clusters are involved in the spontaneous breakdown of mtDNA leading to petite mutants and in the transmission of mtDNA to the progeny. In the present study we examined other yeast species in the genus Saccharomyces for the organization and transmission capacities of their mtDNAs. One group of yeasts, Saccharomyces sensu stricto, have mtDNAs that are larger than 60 kilo bases (kb) and contain many G-C clusters. The other group, Saccharomyces senso lato, contains smaller mtDNAs, ranging in size from 23 to 48 kb, and has almost no G-C clusters. The lower inducibility of petites arising in the

18. Mitochondrial Genetics Genetic characterizations of mitochondria using RFLP analysis show that the phenotypic with a pure Arabidopsis or a recombined mitochondrial genome containing http://www.vbsg.slu.se/eng/research/projects/files_proj/KGlimelius_CMS/CMS.html

Regulation of floral organogenesis and CMS in Brassica Brassica napus Arabidopsis thaliana somatic cybrids as a tool for investigating floral development and cytoplasmic male sterility (CMS) From protoplast fusions between Arabidopsis thaliana and Brassica napus (oil seed rape) alloplasmic lines have been produced. Several lines display modified and aberrant flower phenotypes with distorted petals and anthers. Most of these lines are also male sterile. Genetic characterizations of mitochondria using RFLP analysis show that the phenotypic modifications and male sterility are associated with a pure Arabidopsis or a recombined mitochondrial genome containing mtDNA from both species. Recurrent backcrosses are performed to obtain a stable nuclear Brassica napus genome. The aim is to find mitochondrial genes involved in the CMS trait and flower development. The availability of the complete Arabidopsis thaliana mitochondrial DNA sequence facilitates studies of mitochondrial gene expression by run-on and Northern experiments. We are also screening the material for fertility restorer genes. Such genes are expected to be found in hybrids with parts of the

19. Flower Development & Mitochondrial Genetics Lines displaying modified and aberrant flower. phenotypes with distorted petals and anthers. Normal Brassica napus. CMSline with shrunken anthers. http://www.vbsg.slu.se/eng/research/projects/files_proj/KGlimelius_CMS/lines.htm

Lines displaying modified and aberrant flower phenotypes with distorted petals and anthers Normal Brassica napus CMS-line with shrunken anthers CMS-line with anthers deformed to carpels Uppdaterad

20. Current Research Projects - Neurology, Neurobiology And Psychiatry - University Mitochondrial Research Group. Project Title Mammalian mitochondrial genetics heredity and heteroplasmy. http://www.ncl.ac.uk/nnp/research/mrg/curproject/currproj-6.html

University of Newcastle Neurology, Neurobiology and Psychiatry Research Mitochondrial Research Group ... Neurology, Neurobiology and Psychiatry Search Home Site Map Advanced Search Undergraduate ... External Web Links printable version Mitochondrial Research Group Project Title: Mammalian mitochondrial genetics: heredity and heteroplasmy Principal Investigators: Patrick Chinnery Douglass Turnbull Robert Lightowlers Staff: Joanna Elson Rob Taylor Denise Brown Geoffrey Taylor Collaborators: Neil Howell (UTMB, Texas USA) Henrik Dahl and David Thorburn (Murdock Institute, Melbourne, Aus) David Samuels (Newcastle, UK) Overview: The mitochondrial genome (mtDNA) is a relatively small (16.569bp) circular genome found in multicopy in the mitochondria of all human cells. When a mutation occurs, it will be present in a vanishingly small proportion of the total mtDNA content but will occasionally become fixed in an individual by processes that are not fully understood. Further, if the mutation is pathogenic, it is difficult to predict whether this mutation will be transmitted from the mother to her offspring, what amount of mutated mtDNA will be transferred and how the mutated mtDNA will segregate during development. It is our goal to understand these processes so that we will eventually be able to provide genetic counseling for women who harbour pathogenic mtDNA mutations.

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